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SARCOMA ADVISORY GROUP (SAG) CLINICAL GUIDELINES AND OPERATIONAL POLICY The group agreed to adopt the Guidelines for the Management of Soft Tissue Sarcomas – Robert Grimer et al, Hindawi Publishing Corporation, Sarcoma, Volume 2010 (2010), Article ID 506182, which were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group. The guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) were used as the basis for discussion. Local operational details are included in this document Status: Final Ratified by: EM Sarcoma Cancer SAG Chair – Mr R Ashford 14th October 2011 Endorsed by: Dr Chris Kenny, Chair of the East Midlands Cancer Network 16th November 2011 Review date: June 2012 Distributed to: All Trust Management Teams and Directorates EMCN Sarcoma Management Guidelines – Updates 30 11 11 1 Page Reference Number for Peer Review Measure Measure Number 11-1C-105l 11-1C-106l 11-1C-107l 11-1C-108l 11-1C-109l 11-1C-111l 11-1C-112l 11-1C-113l EMCN Sarcoma Management Guidelines – Updates 30 11 11 Page Number 5 5 9 9 26 45 45 10 2 Contents Contents Page 1.0 2.0 3.0 4.0 5.0 6.0 4 4 5 9 10 14 Introduction EMSS aims Presentation Pathway Diagnostic Pathway Shared Care Pathway - Sarcomas arising at different sites Imaging guidelines - Bone sarcoma - Soft Tissue Sarcoma - MRI protocol - Advice for referring centres 7.0 MDT meeting 8.0 Biopsy & STS specimen reporting - Staging - Prognostic Scoring 9.0 The Treatment Pathway Surgery - Surgical Intent - Retroperitoneal Sarcomas - Radiotherapy - Chemotherapy 10.0 Palliative care 11.0 Rehabilitation 12.0 Patient information & support 13.0 Follow-up Appendix 1 – Soft Tissue Sarcoma Pathway Bone Sarcoma Pathway Soft Tissue Sarcoma Pathway – TYA Soft Tissue Sarcoma Pathway - TYA EMCN Sarcoma Management Guidelines – Updates 30 11 11 19 20 26 41 41 43 45 48 49 3 1.0 Introduction 1.1 Context The Improving Outcomes Guidance (IOG) for people with sarcoma was published by NICE in March 2006. The Department of Health requested that Specialised Commissioning Groups (SCGs) oversee the implementation since the guidance relates to rare cancers and will necessitate supra-network specialisation to meet the population recommendations for a soft tissue service of 2-3 million and for a bone sarcoma service of 7-8 million. The key recommendations from this document as followed by the East Midlands Cancer Network:• All patients with a confirmed diagnosis of bone or soft tissue sarcoma should be treated by a specialist multidisciplinary team (MDT) • Cancer Networks should arrange diagnostic services for the investigation of patients with suspected soft tissue sarcomas at designated diagnostic clinics. All patients with a probable bone sarcoma should be referred directly to a bone treatment centre for diagnosis and management • A soft tissue sarcoma MDT should meet minimum criteria and manage at least 100 new patients per year. A bone sarcoma MDT should manage a minimum of 50 new patients per year • All patients with a provisional histological and/or radiological diagnosis of bone or soft tissue sarcoma should have their diagnosis reviewed by a specialist sarcoma pathologist and/or radiologist who are part of a sarcoma MDT. Commissioners should fund a formal system for second opinions and review of difficult cases and molecular and cytogenetic facilities • Patients should undergo definitive resection of their sarcoma by a surgeon who is a member of a sarcoma MDT or by a surgeon with tumour site specific or age appropriate skills in consultation with the sarcoma MDT • Chemotherapy and radiotherapy are important components of the treatment of some patients and should be carried out at designated centres by appropriate specialists as recommended by a sarcoma MDT 2.0 East Midlands Sarcoma Service (EMSS) Aims EMSS adopts the following principles in patient management:• All treatment decisions must be tailored to the individual patient EMCN Sarcoma Management Guidelines – Updates 30 11 11 4 o The patient should be informed, involved and supported at all times • Cure should be attempted when possible • Potential treatment related morbidity should always be appropriately balanced against the likelihood of cure • Maximise function at all times • The opinion of all members of the MDT should be considered for each patient These aims are written in recognition of the fact that each patient presenting with STS is unique and although treatment decisions usually follow standardised protocols this is not always the case. We know that the treatment of STS can be functionally debilitating when potentially curative interventions are undertaken. Despite treatment with curative intent many patients will relapse with subsequent life limiting disease. Knowledge of the natural history of the disease in question, its treatment, the likelihood of therapeutic success and the informed wishes of the patient are essential in decision making. Current international opinion in the management of localised STS argues that overaggressive local control at the expense of function is more inappropriate than local failure with preserved function. This is counter to established oncological surgical principles and must be underwritten with the caveat that the extent of local therapy must be appropriate in the context of the anticipated natural history of the individual patient’s disease, the individual patient’s rehabilitative potential and any options for further local therapy on relapse. 3.0 Presentation/Referral Pathway (Demonstrating Compliance with Measure 11-1C-105l, 11-1C-106l) 3.1 For Soft Tissue Sarcomas The following referral/ presentation pathway for soft tissue sarcomas of the limbs and trunk wall deals with the pathway of referral from all aspects of primary care to the sarcoma diagnostic clinics. It also covers the pathway of referral to the clinic when a patient presents to a hospital doctor who is not a member of a sarcoma MDT or part of the diagnostic clinic. It covers the referral of newly presenting patients and patients presenting with symptoms suggestive of recurrence. An area wide presentation pathway for soft tissue sarcomas of the limbs and trunk wall has been developed – Appendix 1. Patients should be referred urgently if they present with the following:• A patient presents with a palpable lump that is:o o o o o Greater than about 5cm in diameter Deep to fascia, fixed or immobile Increasing in size Painful A recurrence after previous excision A patient who presents with symptoms suggesting soft tissue sarcoma should be referred to a team specialising in the management of soft tissue sarcoma. Newly presenting patients should be referred on the two week wait:- EMCN Sarcoma Management Guidelines – Updates 30 11 11 5 Trust Kettering General Hospital Northampton General Hospital UHL Derby Hospitals Burton Kings Mill NUH United Lincoln Named Contact 2ww Office Telephone/email 01536 493303 2ww Office 01604 544235 Cancer Office Via Choose & Book Patient Access Centre Choose and Book Helen Andrews Julie Miller 0116 250 2543 Direct Fax 01332 789157 Direct Fax 01283 593090 01623 622515 0115 9691169 01522 512512 Extn 2660 Patients with symptoms suspicious of recurrence should be referred to the diagnostic clinic or a core member of the Sarcoma MDT – details below . Name Role Base Secretary Contact Number Fax Mr Rob Ashford LRI 0116 258 5325 0116 258 6198 NCH 0115 8405883 LCH 0115 9691169 Ext 56872 0116 258 6217 Ext 0115 9691169 Ext 56428 0115 9691169 Ext 54364 0115 9691169 Ext 57300 01522 572233 RDH 01332 786447 01332 783155 NCH 0115 9691169 Ext 59816 0115 9628027 LRI 03003031573 Ext 6295 0116 258 6582 0116 2585942 Dr Salli Muller Consultant Surgeon NSSG Chair Consultant Pathologist (MDT Lead) Consultant Clinical Oncologist Consultant Plastic Surgeon Consultant Plastic Surgeon Consultant Clinical Oncologist Consultant Clinical Oncologist Consultant Clinical Oncologist Consultant Medical Oncologist Consultant Clinical Oncologist Consultant Medical Oncologist Consultant Pathologist Dr Cathy Richards Consultant Pathologist LRI 0116 258 6582 0116 2586585 Dr Zjolt Hodi Consultant Pathologist NCH 0115 9627768 Dr Asma Haider Consultant Pathologist LRI 0115 9691169 Ext 56871 0116 258 6582 Dr Julia Fairbairn Consultant Radiologist NCH 0115 9267776 Dr Winston Rennie Consultant Radiologist LRI 0115 9691169 Ext 55805 0116 258 6898 Dr Tom McCulloch Dr Claire Esler Mr Graeme Perks Miss Anna Raurell Dr Mike Sokal Dr Zuzana Stokes Dr Mojca Persic Dr Ivo Hennig Dr Kate Cardale Dr Samreen Ahmed LRI NCH NCH NCH 0116 2585942 0115 9627939 0115 9627939 0115 8405879 01522 572213 NCH LRI EMCN Sarcoma Management Guidelines – Updates 30 11 11 0116 2583489 0116 2583489 0116 2585584 6 Dr Cheika Kennedy Consultant Radiologist NCH Dr Suchi Gaba Consultant Radiologist LRI Anita Pabla LRI Nita Pringle Macmillan Sarcoma Nurse Clinical Nurse Specialist MDT Coordinator Miss C O’Mara, NUH MDT Co-ordinator Nicola Wilshaw 0115 9691169 Ext 55805 0116 258 6898 0115 9627776 0116 2585942 LRI 03003031573 Ext 7420 0115 9691169 Ext 55605 0116 258 6721 NUH 0115 9249924 NCH 0116 2585584 0116 258 6047 Referral route 1. Uncharacterised soft tissue mass with suspicious clinical features Established risk factors that should prompt suspicion for malignant versus benign soft tissue masses are:• • • • • Size > 5 cm Deeply located (with respect to investing fascia) Rapid growth Symptomatic Recurrence after previous resection The presence of one or more the above should prompt urgent referral via the ‘fast-track’ cancer waiting time (CWT) process. Patients will be seen within 14 days. Ultrasound imaging based triage will be performed locally. Patients may be reassured and discharged if imaging is not concerning without need for review by EMSS. Verbal and written patient information should be provided and referral made using the referral proforma. 2. Uncharacterised soft tissue mass without suspicious clinical features The likelihood of benign disease increases with smaller tumours that are unchanging. Reducing the ‘size cut-off’ may allow earlier detection (and better outcomes) for sarcomas but increases the probability that the tumour will be benign. This may have an impact upon patient anxiety and inconvenience and EMSS capacity to deal with the specialist requirements of true sarcomas. The following clinical features would tend to support a benign diagnosis. • Less than 5 cm and superficial to deep fascia • Local inflammatory change • History and consistent signs of local trauma • Documented no change in size over 6 weeks • Presence of pathognomic features (punctum, transillumination, tenderness) • Associated comorbidity predisposing to benign masses (such as inflammatory arthritis) EMCN Sarcoma Management Guidelines – Updates 30 11 11 7 If a benign diagnosis is suspected but confirmation is required a non-urgent referral is required. Patients will be seen within 28 days of referral. The process described as per (1) will otherwise be followed. 3. Soft tissue masses that may be nodal Soft tissue sarcomas very rarely involve nodal masses. Suspected nodal masses arise most commonly in the context of malignancies of the head & neck, breast, pelvic, cutaneous squamous carcinoma and melanomas, lymphoma, occult/unknown primary carcinomas, lymphomas and benign reactive or inflammatory conditions. Patient with suspected nodal masses should prompt a systematic review of symptoms, clinical examination and fast-track referral to the most appropriate site specific team for work-up rather than referral to the EMSS. An FNAC is inadequate to exclude soft tissue sarcoma or lymphoma and a core biopsy is required. Masses referred to the sarcoma service that are subsequently found to be nodal may result in delayed site-specific management. 4. Suspicious soft tissue mass following imaging Patients whose images are entirely consistent with benign disease do not need to be referred to the EMSS. Referral to the EMSS is needed if imaging has shown:• • • Lipoma requiring further evaluation Indeterminate features Possible sarcoma Radiological criteria are listed in the imaging guidelines. Referral to the EMSS should be made via the EMSS MDT coordinator and requires completion of a referral proforma or a comprehensive letter detailing the patient history and image transfer of appropriate images for review. Failure to provide required supporting material may delay subsequent management. 5. Suspicious histology Inappropriate biopsy and/or resection of soft tissue sarcoma by a non-specialist team are associated with worse outcomes (tumour control and function). For cases when biopsy/resection has been performed and sarcoma is only suspected following histological review (so called ‘whoops’ procedures) EMSS review is advised. Referral to the EMSS should be made via the EMSS MDT coordinator and requires completion of a referral proforma or a comprehensive letter detailing the patient history and transfer of appropriate images and tissue blocks and slides. Written operative notes, radiology and histology reports from the referring team are also required. Failure to provide required supporting material may delay subsequent management. 6. Third party referrals from recognised cancer centres Patients whose diagnostic pathway and/or initial treatment have been started at another recognised sarcoma centre may be referred to EMSS for further management. A written summary of management to date is required rather than a proforma. Histology review is not usually required but full reports are needed. Provision of imaging including written image EMCN Sarcoma Management Guidelines – Updates 30 11 11 8 reports as well as operative notes (when performed) is essential. Image review may be required for assessment of treatment response and/or baseline status for follow-up. 3.2 For Bone Sarcomas The East Midlands Sarcoma Advisory Group in consultation with the MDT agree an areawide presentation pathway for bone sarcomas which specifies:• • • • • • • That patients with X-rays or other images (including incidental findings) which are thought to be possibly indicative of a primary bone sarcoma, should be referred directly to the bone sarcoma MDT. The majority go to the supra-regional service based at the Royal Orthopaedic Hospital (ROH) in Birmingham. A small number of patients from Northamptonshire go to Oxford. That patients with clinical symptoms or signs suspicious of a primary bone sarcoma should be referred directly to the bone sarcoma. That patients diagnosed post-operatively with a previously unsuspected bone sarcoma should be referred directly to the bone sarcoma MDT. The contact number for referral to the bone MDT at Royal Orthopaedic Hospital, Birmingham is Lin Russell, Consultant Nurse, Royal Orthopaedic Hospital, Birmingham – Tel No 0121 685407, Fax 0121 6854146 Biopsy of suspected patients should only be carried out by the bone sarcoma MDT All small cell sarcomas should have molecular/cytogenetic testing Contact points for primary care/hospital doctors to refer back known patients with symptoms suspicious of recurrence The presentation pathway for bone sarcomas is included in Appendix 2 4.0 The Diagnostic Pathway (Demonstrating Compliance with Measure 11-1C-107l, 11-1C-108l) 4.1 For Soft Tissue Sarcomas Route of referral depends upon the level of clinical suspicion and the investigations performed up to the point of referral. Soft tissue masses SHOULD NOT be biopsied without prior discussion with the EMSS MDT. However, the following investigations should be carried out by the local diagnostic clinic prior to referral to the sarcoma MDT:Trust United Lincoln Nottingham University Hospitals Sherwood Forest Royal Derby University Hospitals of Leicester Kettering General Hospital Northampton General Hospital Services Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignancy Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignancy Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignancy Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignancy Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignant Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignancy Physical examination, radiology – ultrasound and MRI, chest X-ray for staging if lesion is worrying for malignant EMCN Sarcoma Management Guidelines – Updates 30 11 11 9 The following diagnostic pathway is for the investigation to establish and confirm the diagnosis, staging and assessment for treatment once the patient has been referred to the sarcoma service. It recognises that some sarcomas with site-specific presentations will be managed according to the agreed shared care pathways. It covers the process for a new diagnosis and for a recurrence. The East Midlands Cancer Network Diagnostic Pathway for Soft Tissue Sarcomas (referred to the sarcoma service) specifies:• That all biopsies should be referred either directly to a specialist sarcoma pathologist. • All small cell sarcomas should have molecular/cytogenetic testing • Imaging modalities and their specific indications • The laboratory and histopathological/histochemical investigations and their specific indications Molecular Biology and Cytogenetics The Cytogenetics Service at Nottingham University Hospitals NHS Trust is the designated laboratory covering FISH for most of the known translocations in sarcoma. The Nottingham Service can also do classical cytogenetics on fresh tissue on the rare occasions this is required. Rt-PCR is sent to the laboratory at the Royal Orthopaedic Hospital in Birmingham GIST additional testing is sent to the laboratory at the Queen Elizabeth Hospital in Birmingham Designated GIST Histopathologists The EMCN SAG has agreed in conjunction with the EMCN Pathology Group that the designated GIST Histopathologists for second opinion and review if required are: Dr T McCulloch, Consultant Histopathologist , NUH Dr Cathy Richards, Consultant Histopathologist, University Hospitals of Leicester These colleagues are members of Sarcoma MDT and Upper GI in NUH and Leicester respectively. 5.0 Shared Care Pathway for Soft Tissue Sarcomas Presenting to Site Specialised MDTs (Demonstrating Compliance with Measure 11-1C-113l This section of the guidance refers to sarcomas arising in the head and neck, gynaecological sarcomas, intrathoracic sarcomas and neurofibromatosis 1-associated sarcomas and urological sarcomas many of which may present to or be partly managed by other site specific MDTs. When this is the case, documented arrangements for linking with the sarcoma MDT must be in place. The East Midlands Sarcoma Advisory Group has agreed with the relevant NSSGs the shared care pathway for soft tissue sarcomas presenting to the site specialist MDTs which includes:- EMCN Sarcoma Management Guidelines – Updates 30 11 11 10 • Which individual MDT or clinic out of the site specialist MDT, sarcoma MDT and specialist clinics should deal with these cases for which parts of the patient’s pathway • Clinical guidelines • All newly presenting cases of soft tissue sarcoma, if not diagnosed initially by a specialist sarcoma pathologist (SSP), should be sent for histological review by a SSP and, in the case of upper GI sarcomas, the histology of all proposed cases of GIST are reviewed by one of the designated GIST pathologists The Shared Care Pathway for STS for the individual sites are set out below:1. Gastrointestinal Stromal Tumours Referral is expected to occur most often at the time of:• Clinical or radiological suspicion of GIST • Biopsy proven or resected GIST The sarcoma MDT will give access to:• Radiological and clinical expertise • Expert pathology including gene mutation analysis • Expertise on use of neoadjuvant systemic treatment • New drugs and clinical trials • Radiofrequency thermoablation and other minimally invasive techniques • Specialist surgery such as hepatic resection • Specialist key worker, information and support It is recommended that all patients should be reviewed by a sarcoma MDT at the time of diagnosis. Surgery for localised tumours may be undertaken in a local referring centre with appropriate surgical expertise and after agreement with the sarcoma MDT. All patients should be reviewed with pathology and imaging as above. It is recommended that systemic therapy will be initiated only after review by the sarcoma MDT. Systemic treatment will be administered within clinical trials where these exist or according to agreed guidelines and will be managed by the sarcoma MDT. Follow up will be in accordance with national guidelines. 2. Gynaecological Sarcomas These sarcomas are often diagnosed after hysterectomy. All patients with suspected or proven gynaecological sarcomas must be discussed with, and usually referred to, the sarcoma MDT. It is anticipated that surgery will be undertaken by members of the gynaecological oncology MDT either at the referring centre or sarcoma centre. The sarcoma MDTs will manage systemic treatment, including hormonal therapy, and radiotherapy for all pure sarcomas (leiomyosarcoma, endometrial stromal sarcoma, rhabdomyosarcoma). MDTs will be managed by the gynaecological oncology team. EMCN Sarcoma Management Guidelines – Updates 30 11 11 11 Management will be undertaken in accordance with guidelines agreed across the two sarcoma MDTs). 3. Head and Neck Sarcomas Sarcomas arising in the head and neck are associated with poorer outcomes than those at other sites. Management is complex and benefits from an experienced MDT. There may be a greater role for neo-adjuvant treatment for sarcomas in this site, requiring close coordination between surgeons and oncologists. All head and neck sarcomas should be referred at the time of suspicion or biopsy. Management will be agreed after joint discussion between the head and neck sarcoma MDT and the Head and Neck cancer MDT at the referring centre. Place of surgery will be advised through this joint treatment planning process and in discussion with referring Head and Neck teams. Management will be undertaken in accordance with guidelines agreed across the two sarcoma MDTs. 4. Thoracic Sarcomas All patients with suspected or proven thoracic sarcomas should be referred to the sarcoma MDT. This includes patients with primary or metastatic disease. Referral is advised at the earliest suspicion of a thoracic sarcoma and assessment, imaging and biopsy will be undertaken by the sarcoma MDT. All patients will be discussed at the joint thoracic sarcoma MDT. All surgery for primary thoracic sarcomas will be undertaken at Glenfield Hospital Leicester or Nottingham City Hospital. All metastectomies will be undertaken at either Glenfield Hospital Leicester (Mr D Waller) or Nottingham City Hospital (Mr J Duffy). Palliative procedures such as pleurodesis may be undertaken elsewhere after agreement by the thoracic sarcoma MDT. 5. Spinal and Intracranial Sarcomas All patients with suspected or proven spinal or intracranial sarcomas must be referred to the sarcoma MDT. Management will be undertaken in conjunction with spinal surgeons at Leicester Royal Infirmary or Queens Medical Centre, Nottingham and neurosurgeons at Queens Medical Centre, Nottingham. Detailed guidance for the management of spinal sarcomas will be developed by the sarcoma MDTs. 6. Skin Sarcomas EMCN Sarcoma Management Guidelines – Updates 30 11 11 12 Sarcomas arising in the dermis are rare. Subcutaneous sarcomas occur more commonly and should be managed by the sarcoma MDT as for other extremity and truncal sarcomas. The sarcoma MDT will be informed of all new skin sarcomas, excluding Kaposi’s sarcoma, including details of the pathology and treatment undertaken. The sarcoma MDT will review all new cases except fully resected Dermatofibrosarcoma Protuberans and will review all recurrences. Management will be undertaken in accordance with guidelines agreed across the two sarcoma MDTs. 7. Breast Sarcomas The sarcoma MDT will review all cases of breast sarcoma. It is anticipated that surgery will be undertaken by local breast services after discussion with the sarcoma MDT. Management will be undertaken in accordance with guidelines agreed across the two sarcoma MDTs. 8. Sarcomas associated with Neurofibromatosis 1 Sarcomas may arise in patients with known NF-1, often under the care of the regional NF service. Sarcomas may also arise in patients with previously unrecognised NF-1 or those not under follow up by a specialist NF service. It is recognised that NF-1 associated sarcomas present specific complexities for management. All NF-1 associated sarcomas will be referred to a sarcoma MDT. Surgery will generally be undertaken by core surgical members of the sarcoma MDT. The sarcoma MDT will manage systemic treatment and radiotherapy. A dedicated forum for discussion of patients with NF is being developed with the support of the sarcoma MDTs. This is currently a monthly meeting. EMCN Sarcoma Management Guidelines – Updates 30 11 11 13 6.0 Imaging guidelines 6.1 Bone sarcoma Bone sarcoma is a radiological diagnosis. Suspected bone sarcoma should be referred directly to a specialist bone sarcoma MDT. EMSS is not recognised as a bone sarcoma centre and referral of bone sarcomas to EMSS may delay referral for definitive treatment. 6.2 Soft Tissue Sarcoma Diagnosis 1. Patient attends GP. 2. Soft tissue Mass present and assessed clinically; a. Observe/Discharge b. Investigate i. 2 week ii. 4 week 3. Ultrasound a. Performed/supervised by clinician – FRCR/ RCR accredited to perform/report ultrasound (preferably MSK but not vital). b. History re-taken regarding – duration, precipitants, growth, associated symptoms. c. Examined for position and local changes. d. Ultrasound machine used must be of diagnostic/medical standard with at least 6 monthly QA of electrical safety, transducer, machine and monitor quality. e. Ultrasound examination to assess – mass size, mass location (relation to fascia), f. Echotexture, cyst/solid/mixed, Doppler characteristics. g. If diagnostic for non sarcoma (benign) (table 1) – report to GP. h. If diagnostic for non sarcoma (malignant) by history and appearances – report to GP to refer to local oncology. i. If diagnostic for lipoma but concerning symptoms or indeterminate and concerning symptoms – grade III lesion (table 2) – report to GP & MRI (notify sarcoma service). j. If suspicious for sarcoma grade IV or V (table 2)–report to GP& MRI (notify sarcoma service). 4. MRI – performed within 2 weeks of Ultrasound (ideally <10 days) a. Recommended protocol – Protocol 1. b. If claustrophobic refer to sarcoma service with ultrasound. c. If diagnostic for non sarcoma (benign) (Table 1) – report to GP. d. If diagnostic for non sarcoma (malignant) (Table 2) – report to GP to refer local oncology. e. If suspicious for sarcoma or indeterminate (Table 3)–report to GP& MRI (notify sarcoma service). 5. Sarcoma Service a. Review MR and US. b. Keep biopsy appointment or c. Postpone biopsy and advise MDT review first 6. Continue on diagnostic pathway. Biopsy 1. Performed by sarcoma MDT member and send to sarcoma histopathologist. EMCN Sarcoma Management Guidelines – Updates 30 11 11 14 2. Image guided – dependant on anatomical location and expertise will be either ultrasound, fluoroscopically or CT guided. 3. Non-image guided (in clinic) - dependant on anatomical location and expertise, may be preferential for expediting management decisions if radiology shows no requirement for targeting focal areas of the mass. 4. Samples should be obtained using a core needle and as large as possible (typically greater than 16 gauge) but will ultimately depend on location, lesion type and patient comorbidity. 5. At least 2 samples should be sent preserved to histopathology and 1 sample fresh to cytogenetics if possible – variations on the need for further fresh specimens will depend on level of suspicion for differing suspected tumour types (see relevant guidelines – link e.g. lymphoma). Staging 1. Primary (local) staging will be typically addressed by the diagnostic ultrasound and/or MRI. 2. Staging CT thorax will be performed routinely to address metastases for highly suspicious lesions prior to known biopsy results or after positive biopsy results. 3. Routine CT/MRI liver, bone or PET scanning is unnecessary except for specific histological subtypes (see relevant guidelines). Relapse 1. Suspected early post treatment complications (< 4 weeks) can be initially assessed by ultrasound to detect abscess, seroma and haematoma. 2. Suspected tumour persistence or tumour recurrence will be typically best evaluated by MRI (may need contrast, see MRI Protocol). Alternative or additional use of ultrasound, CT, isotope bone scan and PET scanning will depend on the suspected tumour type or contraindications to MRI. 3. Biopsy may be necessary to clarify the diagnosis (same criteria as above). MRI protocol guidelines. 1. Mark mass with capsule(s). 2. MR imaging performed in 2 orthogonal planes, 5-6 mm slice thickness. a. Axial and Sagittal or Coronal b. T1 weighted (without fat suppression) c. T2 weighted with fat suppression 3. Fast spin echo sequences can be used to reduce motion artefact. 4. Gadolinium useful a. To determine solid or cystic b. Identify necrotic tumour or haematoma c. Patients who have had previous surgery or radiotherapy 5. If gadolinium given T1 fat saturated sequences should be performed post injection. It is NOT necessary to perform T1 fat saturated sequences pre gadolinium injection. 6. Small field of view is ideal but if very focussed should perform localiser views so subsequent clinicians involved can determine exact body positioning. EMCN Sarcoma Management Guidelines – Updates 30 11 11 15 Follow-up See section on Follow Up Table 1. Benign diagnoses Category Name Description 1 Normal No abnormality seen on ultrasound 2A Benign Cyst Oval lesion, hypo-echoic centrally with a well defined wall and posterior acoustic enhancement 2B Benign Vascular Lesion Solid or cystic structure with minor linear vascularity demonstrated on colour or power settings 2C Benign Other Any lesion with either inflammatory characteristics or benign soft tissue mass. This includes lipomata: Homogenous hyper-echoic lesion within the dermis or deep fat planes, no flow within it on colour or power settings and causing no or minimal mass effect to the surrounding structures. Table 2. Indeterminate and Sarcoma Category Name Description 3 Indeterminate Lipoma with atypical features (vascularity, deep to deep fascia) or other lesion that is (i)Clinically painful or (ii) enlarging solid mass and no Doppler flow 4 Probably malignant Solid, heterogeneous lesion with distortion of surrounding anatomy and disorganized vascularity on doppler flow 5 Malignant EMCN Sarcoma Management Guidelines – Updates 30 11 11 16 Imaging guidelines for suspected soft tissue sarcoma a) Extremity soft tissue mass: Suspected sarcoma on clinical grounds; Soft tissue mass of increasing size, or a lesion > 5cm, or deep to the fascia - 1. Ultrasound triage: ? benign v malignant – non-sarcoma v suspected sarcoma. Ideally performed by musculoskeletal radiologist. Suspicious or indeterminate features warrants MRI (see attached sequence protocol) for lesion characterisation +/- local staging . 2. Plain radiograph of lesion - 3. MRI to be undertaken by Sarcoma musculoskeletal radiologists; Dr K J Fairbairn (NUH) or Dr W Rennie (LRI) If suspicious features refer for discussion at sarcoma MDT 4. Biopsy; ideally should be undertaken by the sarcoma interventional radiologist, Dr C Kennedy (NUH) or Dr S Gaba (UHL) after MR imaging to permit a truly targeted biopsy, usually ultrasound guided. NB – Biopsy pre-MR may complicate appearances due to intra-lesional haemorrhage. Two ultrasound biopsy lists are available each week at NUH – Thursday p.m. and Friday a.m. One ultrasound biopsy list is available at UHL. The sample should be taken directly to the laboratory for attention of the sarcoma Histopathologist – Dr T McCulloch/Dr Z Hodi (NUH) or Dr C Richards/Dr S Muller/Dr A Haider (UHL). 5. Staging; should be deferred until the biopsy result is known. Over 40% of referrals to the EMSS in 2009 were benign or non-sarcomatous diagnoses. Ionising radiation regulations (IR(ME)R) cannot permit unnecessary X-ray exposure in order to expedite throughput. CT Chest will suffice for most STS. Non-contrast, 2.5mm acquisitions. Exceptions: small round blue cell tumours, myxoid liposarcomas, retroperitoneal tumours. Problem solving further imaging will be addressed by the sarcoma radiologists, recognising that visceral metastases are uncommon in most sarcoma subtypes and that PET-CT does not yet have an established role in sarcoma imaging, due to the frequency of low grade tumours, and thus low metabolic activity below the resolution of PET. Whole body diffusion weighted MRI may be of value. b) Retroperitoneal soft tissue sarcoma This tumour group is often asymptomatic and usually an incidental finding following investigation for other symptoms or an unexpected diagnosis post surgical resection. Baseline CT chest, abdomen and pelvis post oral and intravenous contrast for tumour characterisation, assessment of resectability and staging will be required prior to MDT review. Biopsy as above, should ideally be performed by a sarcoma radiologist – Dr C Kennedy (NUH) - after image review with a retroperitoneal surgeon at the Sarcoma MDT – Mr M Robinson. All patients will require 4 hours recovery in a day case bed post retroperitoneal biopsy, due to delayed presentation of retroperitoneal haemorrhage. EMCN Sarcoma Management Guidelines – Updates 30 11 11 17 Exception: Well differentiated fatty retroperitoneal lesions do not warrant pre resection biopsy as are pathognomonic of well-differentiated liposarcoma. SPECIAL CASE: Radiological/histopathological discordance If the resulting histopathology is discordant with the suspected radiological tumour type, then re-discussion at MDT should ensue. This is particularly to be emphasised if a benign result is obtained in clinical or radiologically suspected malignancy OR if the biopsy was clinical rather than image guided. The MDT will decide whether to re-biopsy using image guidance or for excision biopsy . INVESTIGATION STRATEGY FOR PRESUMED SOFT TISSUE SARCOMAS: ADVICE FOR REFERRING CENTRES 1. Clinical assessment of lesion 2. Plain radiograph of the relevant area should be obtained 3. Ultrasound triage may be performed if felt clinically indicated to exclude obvious benign lesions eg lipoma. 4. MRI a. Skin markers should be placed above and below the lesion b. Surface coils if the lesion is appendicular. FOV to cover the entire lesion. c. The MRI should be ideally reviewed by a radiologist to ensure the whole lesion is visualised (normal tissue proximally and distally) d. The following sequences should usually be included for presumed STS (See attached RCR recommended sequences) i. T1 and STIR images in at least two planes ii. A T2WSE scan in the best orthogonal plane (usually the transverse plane in the axial skeleton). iii. Contrast enhanced T1W fat suppressed scans would be useful. e. The dimensions of the lesion should be measured in at least two planes f. Further advice is available from the lead Sarcoma radiologists Dr Julia Fairbairn at Nottingham University Hospital City Campus 0115 969 1169 ext 55805 or Dr Winston Rennie at Leicester Royal Infirmary 0116 258 6898. g. In the event that the patient is unable to have an MRI then an USS and CT contrast enhanced should be performed with measurement of the lesion and its density on CT if possible. 5. Staging If the lesion is worrying for malignancy then a chest radiograph can be obtained. Formal staging will be undertaken at the tertiary centre after the histology is known. 6. Biopsy Please DO NOT biopsy the lesion but refer to the regional centre. 7. Upon referral to the East Midlands Sarcoma Service, DICOM images of all relevant imaging should be transferred to the regional centre c/o Stephanie Ferrington, X-ray EMCN Sarcoma Management Guidelines – Updates 30 11 11 18 department, City Campus, NUH, along with relevant imaging reports and clinic letters. REFERENCES Recommendations for Cross-Sectional Imaging in Cancer Management. RCR (06)1 UK Guidelines for the Management of Soft Tissue Sarcomas BSG Royal College of Radiologists recommendations for MRI Imaging protocol of STS. RCR (06)1 Sequence Plane Slice thickness Field of view T1W Sag/Cor 6 +/- 2 mm Cover lesion T2W Sag/Cor 6 +/- 2 mm Cover lesion T2W & T1W Axial 8 +/- 2 mm Cover lesion STIR Best plane 8 +/- 2 mm Cover lesion T1W + contrast Best plane 8 +/- 2 mm Cover lesion 7.0 MDT meeting The MDT meeting should facilitate appropriate multidisciplinary discussion of patients referred to or managed by EMSS. Responsibility for the organisation of the meeting lies with the NUH Cancer Centre staff. PPM should be used to schedule an agenda and populate clinical summaries. Discussion requires timely provision of images and histopathology specimens. Clinical background should be available from the referral proforma or a comprehensive letter detailing the patient history or, if already known to the MDT and referred by an EMSS MDT member, via a summary of the question to be addressed entered into an annotation on PPM. The MDT coordinator should be made aware by telephone or, ideally, e-mail ([email protected] or clare.o’[email protected] ). The MDT meeting should be chaired. It is the responsibility of the chair to make an appropriate signed paper entry into the MDT record. A simultaneous ‘live’ entry onto CNODES summarising the MDT discussion will be made by EMSS office staff. The MDT record should make clear the agreed: Radiology results Histology results Action plan Responsibility for action plan Radiology results EMCN Sarcoma Management Guidelines – Updates 30 11 11 19 Radiology reports may have already been issued. When a change to the report is made this should be recorded both in the MDT record and as an addendum to the issued report. Diagnostic studies Should include specific comment about the likelihood of malignancy and the need for further investigations. The size of lesions and relation to fascia should be reported. Staging studies As diagnostic studies. Also to include relationship to neurovascular structures and viscera. Presence or absence of metastatic lesions should be recorded. Treatment response studies Should include specific comment about the date of the baseline study as well as response to therapy according to either RECIST or Choi (GIST only) criteria. Histology results Histology reports may have already been issued. When a change to the report is made this should be recorded both in the MDT record and as an addendum to the issued report. Action plan It is essential that an outcome is recorded for each case discussion. Although free-text will be needed for radiology and pathology comments, most MDT outcomes can be recorded using a limited number of outcome codes. Summary of outcomes Missing data Re-discuss at MDT meeting Update named clinician directly (no need to re-discuss at MDT meeting) Benign Further imaging for interval change Imaging for local treatment planning Excision biopsy Discharge Likely benign Further imaging for interval change Imaging for local treatment planning Excision biopsy Probably malignant Further imaging for diagnosis and staging Imaging for local treatment planning Core biopsy Excisional biopsy Incisional biopsy (exceptional circumstances only) Planned marginal excision Wide local excision Malignant Further imaging for diagnosis and staging EMCN Sarcoma Management Guidelines – Updates 30 11 11 20 Imaging for local treatment planning Core biopsy Incisional biopsy (exceptional circumstances only) Re-excision Wide local excision Planned marginal excision Planned positive excision Radical excision/amputation Palliative surgery Isolated limb infusion or perfusion Systemic therapy Radiotherapy Best supportive care Non-Sarcoma Malignancy – Refer to Other MDT Responsibility for action plan The MDT recommendation may have been made without face-to-face clinician review of the patient. The treating clinician retains the right to modify the MDT opinion based upon his/her assessment of the case-specific clinical features, including co-morbidities and general performance status, following discussion with the patient. Changes should be fed-back to the MDT coordinator. Responsibility for action is assigned based upon submission of a radiology request, biopsy request, an out-patient clinic booking or a specifically addressed letter. An appropriate timescale should be indicated where necessary. Radiology or biopsy submissions should generate an MDT discussion following the requested examination. MDT meeting letters To aid communication and to support responsibility for action planning, a summary annotation or formal letter should be sent to involved clinicians following the MDT meeting. 8.0 Biopsy & STS specimen reporting Introduction This section supplements the Data set for Cancer Histopathology Reports on Soft Tissue Sarcomas, which is under consultation with the Royal College of Pathologists. All soft tissue tumour cases will be selected for review as per the national and local guidelines. There are five specialist sarcoma pathologists in the regional network reporting the specimens, one of whom is the nominated lead pathologist. They will contribute to the regional sarcoma MDT, participate in the National Soft Tissue MDT and in network and local audit. All patients with soft tissue tumours assessed in a diagnostic clinic should have their pathology reported by a specialist soft tissue pathologist. Specimen Types EMCN Sarcoma Management Guidelines – Updates 30 11 11 21 Fine Needle Aspiration Cytology Has only very limited role in the diagnosis of soft tissue sarcomas and should be avoided as far as possible. Needle core biopsies The default biopsy. Ideally at least two cores or more should be submitted in formalin. Open biopsies These are recommended only when a core biopsy has been unsuccessful and it is envisaged that the biopsy will definitely inform further management e.g. type of operative procedure. Resection specimens This includes large specimens, amputated limbs, limb girdle amputations, chest wall resections, retroperitoneal sarcomas and sarcomas associated with specific organs. Molecular studies For FISH assessment six unstained sections cut at 1µ and mounted on super frost slides should be sent for FISH analysis. Currently probes are in use for most of the major translocation sarcomas. Further molecular studies can be arranged with outside institutions. Specimens should be reported to an agreed time frame so as to allow appropriate clinical decision making at a planned MDT meeting. Clinical information required on the request form In addition to the demographic data, the following information should be included in the request form. 1. Duration, site, size and plane of the tumour (subcutaneous, intramuscular etc.) 2. History of relevant past illnesses, radiotherapy, chemotherapy or surgery. Surgical intention e.g. planned marginal excision, wide excision etc. Preparation of specimen prior to dissection Core Needle Biopsies Ideally, at least two samples should be provided to the histopathology department in formalin for histopathological examination. Open biopsies If the specimen is large enough, small pieces of tissue can be taken for cytogenetics and also to be frozen in liquid nitrogen at 80 degrees centigrade. Resection specimens The specimen should be weighed, inked if this is desirable (NB inking may be deleterious and produce false margins if there are multiple tissue planes excised) and measured. The specimen is then sliced. The main specimen is placed in formalin for adequate fixation. Photograph of the specimen before and after slicing is desirable. In the case of a wide excision for a proven malignancy blocks are taken to include all resection margins macroscopically less than 30mm from tumour and particularly the nearest resection margin. Adequate numbers of blocks should be taken. Areas, which appear visibly different, require adequate extra sampling. In large liposarcomas, particularly of the retroperitoneum, any area with different colour or consistency should be adequately sampled EMCN Sarcoma Management Guidelines – Updates 30 11 11 22 to detect dedifferentiation. In the case of high grade sarcomas any abnormal appearing local fat should be sampled to exclude dedifferenatiated liposarcoma. Core data for soft tissue sarcoma reporting Clinical: Site Plane of the tumour Macroscopic Description: Type of excision Incisional Excisional Radical Maximum tumour dimensions. Measurements should be given in three dimensions. Presence or absence of necrosis and percentage of necrosis. Other features of note. Ossification. Calcification. Microscopic Description: Histological type. Soft tissue sarcomas are categorised based on WHO consensus classification of 2002. Grade (FNCLCC) 1 2 3 Immunohistochemistry results. Tissue planes involved cutaneous subcutaneous deep fascia subfascial intramuscular Status of margins involved marginal wide radical Cytogenetics or Molecular studies. SNOMED codes Pathologist: Date: EMCN Sarcoma Management Guidelines – Updates 30 11 11 23 Reporting of small biopsy specimens The report should include the histological diagnosis with grade with the caveat that the excision specimen may have a higher grade. Immunohistochemical results should be included where appropriate. Results of molecular and cytogenetic studies can be issued as supplementary reports. Points to note: 1. Extremity pleomorphic sarcomas with myogenic differentiation have a worse prognosis compared to others. Hence immunohistochemical positivity for myogenic markers such as smooth muscle actin, Desmin, smooth muscle myosin, H-Caldesmon or nuclear positivity for Myogenin or MyoD1 should be specifically noted. The pattern of staining should be mentioned i.e. diffuse, focal or occasional. 2. Retroperitoneal high-grade sarcomas could possibly represent dedifferentiated liposarcomas, which are known to have better prognosis. In order to confirm the latter diagnosis, any fatty tissue surrounding the tumour should be sampled extensively to identify well-differentiated liposarcomatous areas. Immunohistochemical staining for MDM2 or FISH analysis on paraffin blocks will aid in the diagnosis. Referral for review or specialist opinion All patients seen within the East Midlands Cancer Network with suspicion or diagnosis of sarcoma must be reviewed by the Regional Sarcoma Team. The complete histopathology report should be available at the MDT meeting. The reports, slides and blocks should be requested at least five days prior to the meeting and should be available at least three days before the meeting for review. A formal report should be issued by the reviewing pathologist to the clinician responsible for the patient and also to the original pathologist. References Guidance on Cancer Services Improving Outcomes for People with Sarcoma. The Manual. London: National Institute for Health and Clinical Excellence, 2006. American Joint Committee on Cancer. Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002. Pathology appendices A. SNOMED codes B. FNCLCC grading C. Translocations and other genetic abnormalities in sarcomas Staging Preoperative staging should be completed for all tumours using the current version of the UICC TNM staging system. This may need to be updated based upon operative findings. Tumour (T) EMCN Sarcoma Management Guidelines – Updates 30 11 11 24 There are 3 variables for local tumour extent. Tumour size Tumour relationship to deep fascia Tumour grade Nodes (N) Nodal disease is uncommon in adult type STS but should be carefully looked for in paediatric type STS and selected adult-type STS histological subgroups. Metastases (M) Metastatic disease should be actively sought in all but low-grade primary tumours. Cross-sectional CT imaging of the thorax should be performed as a minimum, with additional series (abdomen/pelvis/neck) performed based upon the site of the primary. Supplementary imaging using alternate modalities may be required. The routine role of PET is unclear. The sites of metastatic disease should be recorded. Prognostic scoring A well validated prognostic scoring tool exists for determining the risk of sarcoma-specific death at 10-12 years post resection for non-metastatic soft tissue sarcoma. This tool may be of value in aiding MDT decision making in respect of balancing the functional consequences of local therapy and in guiding intensity of follow-up. It may also aid patient discussions, but its limitations should be made clear. It is not intended that survival prognosis be forced upon patients who do not wish to receive it. For higher risk patients it may serve as a starting point for discussion of adjuvant systemic therapy, though this is not an approach used routinely at present. Several versions of the tool exist. The original Memorial Sloan Kettering Cancer Centre (MSKCC) tool has been validated for all tumours sites and uses the grading system proposed by Hadju and used by the NCI. Additional tools have been developed to look at limb sarcomas using the FNCLCC grading system used by EMSS and separately for adipocytic tumours which behave markedly differently when either low versus high grade and which represent a greater proportion of retroperitoneal sarcomas. A tool for post-local relapse survival estimation is also available. In time ready-reckoners will be developed to aid more rapid use within the MDT meetings. Four tools are shown: 1. Kattan 2002: all sites, all histologies, NCI grading 2. Dalal 2006: all sites, adipocytic only, NCI grading 3. Mariani 2004: limb only, all histologies, FNCLCC grading 4. Kattan 2003: all sites, post-relapse, NCI grading EMCN Sarcoma Management Guidelines – Updates 30 11 11 25 9.0 The Treatment Pathway (Demonstrating Compliance with Measure 11-1C-109l) The Treatment Pathway for Soft Tissue Sarcomas covers the process of active treatment delivery up to but not including follow up. It covers this process for radical and palliative intent and for treatment of a first presentation or of a recurrence. It covers the situation where the treatment plan is to offer palliative and supportive care only, rather than active tumour removal or cytoreductive therapy. The Treatment Pathway is attached as Appendix 1. The Treatment Pathway includes:• Which team from the sarcoma MDT, teenage and young adults MDT (TYAMDT) or specialist palliative care MDT (SPCMDT) is responsible for which aspects of care. • At which stages in the pathway the patient should be referred between teams. • Clinical Guidelines. • That the treatment planning decision for initial management and for any active treatment of at least any local recurrence and first distant recurrence should be made only after discussion at the sarcoma MDT 9.1 Surgery Surgical intent Surgical intent should be clear preoperatively following MDT work-up and discussion. The extent of surgery should reflect the anticipated future behaviour of the tumour (local and systemic), the place of planned adjuvant therapy, the functional (and cosmetic) impact and surgery-specific risks. • Incisional biopsy Resection of lesional tissue for diagnosis. This is a procedure of last resort (multiple nondiagnostic core-biopsies) in the context of suspected STS. An excisional biopsy may be preferable as residual disease is inevitable. Biopsy should be planned to facilitate enbloc resection of biopsy site when definitive surgery performed. • Excisional biopsy Resection of tumour with narrow margin of normal tissue when either biopsy or imaging is strongly supportive of benign histology. Functional compromise should be minimised. • Wide local excision Resection of tumour surrounded by a non-involved cuff of normal tissue (notionally 2cm; see surgical principles described below). Performed when malignant tumour is proven or suspected on histology or imaging. Functional compromise possible. • Planned marginal excision As WLE but when one or more margin is at higher risk of histological positivity as a wider margin would have a more major impact upon function. EMCN Sarcoma Management Guidelines – Updates 30 11 11 26 • Planned positive excision As planned marginal excision but when tumour is consciously left as resection would have a more major impact upon function. Such margins should be clearly marked and described to facilitate post-operative radiotherapy. Planned positive margin resection is not the same as surgical debulking which should usually be avoided (see palliative surgery). • Re-excision Should aim to convert an unplanned positive excision into a marginal (or ideally a wide) excision by re-excising the tumour bed. Functional compromise is likely. • Radical excision/amputation Radical removal of an entire compartment, limb or part of limb. May be required if tumour crosses several compartments or surgery (and/or tumour) critically disrupts distal neurological or vascular supply in order to attain clear margins. Functional compromise certain. • Palliative surgery Non-curative debulking surgery is not indicated unless there is an over-whelming clinical need such as severe pain, fungation, vascular compromise (haemorrhage or ischaemia) or luminal obstruction. Limb tumours operated upon in this context are usually best managed with an amputation even when metastatic disease is present. Surgical principles Specific surgical techniques remain at the discretion of the operator. Sarcomas are unencapsulated and should be excised en-bloc with an intact cuff of non-compromised muscle, fascial plane, periosteum/bone or organ/viscera. Tumour spillage should be avoided and, if occurs, clearly recorded in the operative notes. The planning of adjuvant radiotherapy is aided if clips are placed at the cranial and caudal extent of the tumour bed and where the surgeon feels that the tumour resection margin is likely to be close and/or involved. The position of clips and any suspected residual (R1 or R2) tumour should be clearly recorded in the operative note. It is also helpful if resected/sacrificed muscles, vessels and/or organs are clearly described. If periosteal stripping is performed this should be recorded. Wound closure Wound closure should be planned, ideally preoperatively, with due regard to received, potential or planned adjuvant radiotherapy. Primary direct closure should be avoided following pre-operative radiotherapy. When post-operative radiotherapy is likely, choice of closure technique should facilitate radiotherapy planning within 4 weeks of surgery and commencement of radiotherapy within 6 weeks of surgery. Skin grafting should be avoided when radiotherapy is anticipated. Surgery - Retroperitoneal Sarcomas Most retroperitoneal sarcomas are liposarcomas or leiomyosarcomas. Guidance below refers to this large majority of non-GIST tumours. Resectable non-metastatic EMCN Sarcoma Management Guidelines – Updates 30 11 11 27 Retroperitoneal sarcomas are often large but not usually infiltrative. Surgery alone is the usual mode of treatment. Most resections are “planned marginal excisions” and often require resection of other adjacent or involved intra-abdominal organs. Local recurrence is common even after extensive surgery. There is no role for routine use of adjuvant RT. Consideration of radiotherapy should only be within the confines of the EORTC trial. Surgery may not be appropriate for elderly patients or those with severe comorbidity, particularly where symptoms are minor and the tumour may be indolent. Unresectable or metastatic Systemic chemotherapy should be delivered as described for limb/trunk. If there is a clear response on imaging surgery can be reconsidered. But there is generally no place for palliative surgery. Inoperable disease post-chemotherapy should be consolidated with radiotherapy. Progressive (and/or metastatic) disease requires palliative dose RT for symptom control only. Pelvic Resectable non-metastatic Uterine sarcomas are managed by the gynaecology MDT. Resection of other pelvic sarcomas should be undertaken by the appropriate site specific team after discussion with the sarcoma MDT. There is no role for neo-adjuvant therapy but post-operative radiotherapy should be considered after review within the sarcoma MDT. Technical RT delivery should be undertaken by the appropriate site specific team. Unresectable or metatstatic Systemic chemotherapy should be delivered as described for limb/trunk. If there is a clear response on imaging surgery can be reconsidered. But there is generally no place for palliative surgery. Inoperable disease post-chemotherapy should be consolidated with radiotherapy. Progressive (and/or metastatic) disease requires palliative dose RT for symptom control only. Retroperitoneal / Abdominopelvic GISTs Operable non-metastatic The primary tumour should be locally excised. Node dissection is not needed. Adjuvant therapy is not indicated. Patients should go onto surveillance based upon their risk of relapse. Inoperable non-metastatic and metastatic disease Treat with first-line receptor tyrosine kinase inhibitor (RTK, currenly imatinib). Following maximal tumour response resectional surgery may be possible. The role of interval debulking for multi-site primarily inoperable disease is unclear. Resection/ablation of local sites of progression on a background of systemic treatment responsive disease may be considered. First line Baseline scan followed by two months of RTK. Repeat imaging and continue with RTK unless objective progressive disease (assess using Choi criteria). Continue with RTK and interval scanning 3 monthly until radiological progression. EMCN Sarcoma Management Guidelines – Updates 30 11 11 28 Options on progression Localised progression only Consider resection/ablation. RTK continues. Widespread/unresectable progression Consider sunitinib. If sunitinib declined consider phase I clinical trials. Dose escalated imatinib (400mg bd) not approved by D&T or NICE. Treatment refractory Withdraw RTK at point of symptomatic progression and deploy symptomatic/best supportive care 9.2 Radiotherapy Role of radiotherapy Postoperative radiotherapy for patients with soft tissue sarcoma allows function preservation with local control rates, and survival, similar to radical resection ( i.e. compartmental excision or amputation). For non-resectable or metastatic disease radiotherapy can be useful in improving localised symptoms and delaying progression. Radiotherapy volumes and doses should be disease and site appropriate according to agreed local protocol but see recommendations below. Radiotherapy for resectable disease Post or pre operative radiotherapy should be used for selected patients following discussion at the regional sarcoma MDT meeting. Indications for postoperative radiotherapy follow European Consensus Guidelines and have recently been supported by British guidelines on the management of soft tissue sarcomas. Nearly all intermediate or high grade soft tissue sarcomas require post operative radiotherapy. Low grade tumours are unlikely to require radiotherapy unless they are large, deep and have been incompletely resected with further surgery likely to compromise function. These guidelines do not apply for retroperitoneal STS or sarcomas arising from viscera when decisions must be made on a case-by-case basis as judged by the risk of local recurrence, the potential gains of radiotherapy and anticipated radiotherapy toxicities. The MDT should discuss and agree local therapy (both primary and adjuvant therapy) prior to surgery to optimise outcomes. Consideration should be given to the functional deficits and complications expected from all modalities used for local control. Placement of surgical clips, detailed operative notes and all preoperative imaging are essential in planning effective postoperative RT. Patients should be seen by the Clinical Oncologist as soon as possible after surgery to facilitate timely radiotherapy planning (with simulation after operative swelling has settled) in order to start post-operative RT within 42 days of surgery. Occasionally in cases of delayed wound healing radiotherapy will need to be delayed further. When bone that has been stripped of periosteum is irradiated the fracture risk is such that in long-bones prophylactic nailing 8-12 weeks post-RT should be considered. EMCN Sarcoma Management Guidelines – Updates 30 11 11 29 European consensus on adjuvant RT for resected STS Dose and fractionation The recommended post operative radiation dose is 60 – 66 Gy in 2 Gy/#. A two phase technique using a shrinking field is commonly employed; 50 Gy to the initial larger volume followed by 10 – 16 Gy to a smaller volume. This dose may need reducing if the treatment field include critical structures (i.e. spinal cord, brachial plexus). Entry into the Vortex study should be considered for extremity soft tissue sarcomas. This randomised clinical trial is comparing the conventional two phase radiotherapy technique with a single phase plan to a smaller volume in an attempt to spare normal tissue and hence improve future limb function but without compromising local control. Pre operative radiotherapy Pre operative radiotherapy is not routinely used in the UK. However, it may be preferred in certain situations where the size of the radiation field required for post operative treatment is likely to result in significant late toxicity or when it is thought radiotherapy may render a tumour with borderline resectability operable. However, if a tumour is clearly unresectable radiotherapy will not alter the situation. Certain radiosensitive tumours such as myxoid liposarcomas will benefit more than less radiosensitive tumours. Preoperative radiotherapy in limb soft tissue sarcoma is associated with increased post operative complications compared to standard post operative radiotherapy although with less late toxicity. Local control rates are comparable. This needs to be considered when planning surgical closing techniques. Dose and fractionation The recommended dose for pre operative radiotherapy is 50 Gy in 2 Gy/# followed by surgery 6 weeks later. If margins are involved then post operative radiotherapy (10 -16 Gy) is recommended. Radiotherapy for unresectable or metatstatic disease EMCN Sarcoma Management Guidelines – Updates 30 11 11 30 Volume and dose selection should be disease and site appropriate and be made with due consideration of the patient’s general performance status (PS) and anticipated natural history and overall survival. Unless the patient’s PS is poor or the patient is clearly at, or nearing the, very late stages of their illness it is suggested that a high-dose palliative approach is adopted and due consideration given to the fact that soft tissue sarcoma is radio-sensitive but not particularly radio-responsive and that tumour shrinkage may take months (though clinical benefit in terms of symptom relief may come within weeks). 9.3 Chemotherapy Role of systemic therapy in soft tissue sarcoma Although not generally chemosensitive systemic therapy has a very important role in selected STS sarcoma subtypes and in the management of bone sarcomas. Management of bone sarcoma is out with the remit of the EMSS MDT but members of the EMSS NSO team may be engaged to provide local delivery of therapy for bone sarcomas whose overall management is being coordinated by a recognised bone sarcoma centre. Systemic therapy may be used as an adjuvant or palliative therapy. Fitness for treatment All patients considered for systemic therapy should be of acceptable performance status (WHO/ECOG PS 1 (or ‘good 2’)) or better and have adequate physiological and haematological reserve following appropriate safety testing. Treatment supervision Systemic therapy should be supervised by core EMSS MDT members. Multi-agent and/or inpatient regimens should be supervised by a EMSS oncologist. Primary systemic therapy Most STS is poorly chemosensitive. Primary use of systemic therapy is not recommended for ‘down staging’ as part of multimodality treatment approaches unless the tumour is of ‘paediatric type’: Ewing’s Sarcoma Rhabdomyosarcoma (paediatic-type, non-pleomorphic) (Osteosarcoma) (GIST) Non-paediatric type STS which is known to me more chemosensitive (see section below) may be considered for primary chemotherapy if it is felt that the tumour is otherwise unresectable without major functional morbidity. Adjuvant systemic therapy The following subtypes of adult-type STS are known to be more chemosensitive: EMCN Sarcoma Management Guidelines – Updates 30 11 11 31 Synovial sarcoma Leiomyosarcoma Myxoid liposarcoma ‘Adjuvant’ chemotherapy for patients with these subtypes may be considered for fit patients who present with either potentially resectable metastatic disease or who are at very high risk of developing metastatic disease following standard resection and adjuvant local therapy of the primary. Even in the absence of clear data supporting routine adjuvant therapy it is felt that early delivery of systemic therapy to these patients may at least prolong relapse-free survival and maximises likelihood of cure for a small proportion (probably less than 15%) by selecting patients who are shown to be at high risk for future systemic failure, have more chemosensitive subtypes and who are less likely to develop treatment related toxicity (by virtue of low tumour load and better overall fitness). A schema for the management of these patients is presented following ‘Palliative systemic therapy’ Palliative systemic therapy Unresectable local or metastatic STS is usually incurable, particularly if arising as a less chemosensitive subtype. Systemic therapy may be offered as a means of improving symptom control, delaying progression or possibly improving overall survival. Survival gains are likely seen in responding patients only. Palliative chemotherapy should only be given to patients fulfilling fitness criteria and who have completed written informed consent. Baseline recording of index disease should be performed. Clinical review should follow each cycle of therapy with response assessment performed after every 2-3 cycles. Unless responding, patients would not normally receive more than 4 cycles in total. More than 6 cycles should not be given unless as part of a sub-type specific protocol (Ewings, Rhabdo, …). Potentially resectable disease Patients with 3 or fewer resectable lesions should be considered for ‘induction’ chemotherapy followed by resection. A surgical opinion should be sought prior to chemotherapy and, if resectable, the patient should be offered 2 cycles of chemotherapy prior to surgery. Those patients with tumours showing significant necrosis histologically should be offered 2-4 further cycles of post-operative adjuvant chemotherapy. Those with otherwise resectable disease who either decline systemic therapy or for whom systemic therapy is too great a risk should have their surgery deferred and a 3 month interval imaging study performed. If there is no progression (no new lesions) on interval scanning surgery should proceed. Progressive disease should be treated as for palliative. Borderline resectable disease in patients who are fit for chemotherapy should undergo resection and then be considered for ‘adjuvant’ chemotherapy as described previously. Chemotherapy protocols are included on the EMCN website at:www.eastmidlandscancernetwork.nhs.uk/_HealthProfessionals-Chemotherapy-OncologySarcoma.aspx EMCN Sarcoma Management Guidelines – Updates 30 11 11 32 9.4 Disease specific recommendation Malignant soft tissue tumours Adult-type Limb & trunk Non-metastatic Clearly operable (limb preservation) Wide local excision should be attempted in all cases. If WLE feasible based upon preoperative staging consider pre-operative radiotherapy if it is felt that this option will produce less late morbidity than post operative radiotherapy (See radiotherapy section). Preoperative RT should not be used if the lesion is likely low grade or if resection likely to be marginal. If pre-operative RT is to be used, consideration should be given to the method of defect closure and the downstream functional consequences of post-operative wound complications. Consideration should be given to re-excision if margins following primary excision are R1 or R2 (ie. an overall R0 excision should be the goal). If pre-op RT is not used consideration should be given to post-op RT given resected tumour grade, margins, size and relationship to deep fascia. European consensus guidelines should be followed. It is helpful for postop RT planning if surgical clips are placed at the cranial and caudal extent of the surgical bed for limb tumours and in addition laterally for truncal tumours. Borderline operable (limb preservation) If WLE not deemed possible on pre-operative staging, consideration should be given to high risk limb preservation or planned marginal resection. Planned marginal resection A planned marginal resection may carry relapse risk only slightly higher than WLE, but clip placement at the site of anticipated close margins and careful operative and histopathology reporting are needed to guide a higher dose of adjuvant RT. Higher dose RT carries a greater risk of late functional morbidity and this should be considered if this combined modality approach is to be deployed. Pre-operative RT is not known to improve resectability and should not be used as optimal management of positive surgical margins in this context is unclear. Amputation For synovial sarcomas and myxoid liposarcomas chemotherapy response rates are such that neoadjuvant combination systemic therapy with doxorubicin and ifosfamide can be considered. If there is no objective response after 4 cycles of treatment (or if progressive disease is identified at any stage) the patient should proceed directly to surgery. In selected cases isolated limb perfusion may have a role. Post-operative RT is usually required. Functional implications of aggressive CMT should be considered. Prior to amputation consideration should be given to the likelihood of metastatic failure. Metastatic Aggressive local surgery should be avoided in patients with known inoperable metastatic disease. Systemic chemotherapy should be considered. If systemic therapy not deliverable or disease becomes refractory consider palliative radiotherapy. Palliative surgery should be reserved for control of difficult local symptoms. Systemic therapy EMCN Sarcoma Management Guidelines – Updates 30 11 11 33 Patients for systemic therapy should be of WHO PS 2 or better without medical comorbidities that would compromise safe drug delivery. Physiological safety testing is mandatory. All patients require baseline CT and repeat imaging after ideally every second (if not third cycle) . Poor tolerance/excess toxicity in the absence of an objective response of CT should prompt discontinuation. Patients without an objective response should not proceed beyond cycle 4. No patient should receive more than 6 cycles per course. First-line Overall survival with combination chemotherapy (doxorubicin and ifosfamide) is not superior to single agent doxorubicin alone. The toxicity from combination treatment is much higher. With the exception of synovial sarcoma and myxoid liposarcoma single agent doxorubicin is first-line for adult-type soft tissue sarcoma. For the above named sub-types response rates are superior for combination doxorubicin and ifosfamide and this should be considered. In cases where a fast response is desirable or where an attempt is being made to render a patient operable then combination doxorubicin and ifosfamide should be considered. In cases where doxorubicin can not be used for medical reasons, usually cardiac, Trabectedin may be considered as first line treatment. This is most likely to be beneficial in Myxoid liposarcomas. (NICE guidelines 2010) Second-line Single agent ifosfamide should be considered in patients who have not received it as first line. If they have already received ifosfamide or if they are not suitable for ifosfamide for medical reasons they should be considered for Trabectedin or Gemcitabine and Docetaxel. Essentially: Trabectedin should be used Liposarcomas and pleomorphic sarcomas NOS Gemcitabine and doxetaxel should be used for Leiomyosarcomas Other histological types can be considered for either option Trials Available trials should be considered at all times. Referral between centres in the EMCN should be actively encouraged. There will be times when referral to out of region specialist centres is recommended for appropriate trials. Retroperitoneal GIST Operable non-metastatic Primary tumour should be locally excised. Node dissection is not needed. Adjuvant therapy is not indicated. Patients should go onto surveillance based upon their risk of relapse. Inoperable non-metastatic and metastatic disease Treat with first-line receptor tyrosine kinase inhibitor (RTK, currenly imatinib). Following maximal tumour response resectional surgery may be possible. The role of interval debulking for multi-site primarily inoperable disease is unclear. Resection/ablation of local sites of progression on a background of systemic treatment responsive disease may be considered. First line Baseline scan followed by two months of RTK. Repeat imaging and continue with RTK unless objective progressive disease (assess using Choi criteria). Continue with RTK and interval scanning 3monthly until radiological progression. EMCN Sarcoma Management Guidelines – Updates 30 11 11 34 Options on progression Localised progression only Consider resection/ablation. RTK continues. Widespread/unresectable progression Consider sunitinib. If sunitinib declined consider phase I clinical trials. Dose escalated imatinib (400mg bd) not approved by D&T or NICE. Treatment refractory Withdraw RTK at point of symptomatic progression and deploy symptomatic/best supportive care In development Genotyping Non-GIST Resectable non-metastatic Retroperitoneal sarcomas should be discussed at the sarcoma MDT before surgery. Surgery should performed by one of the retropeitoneal sarcoma surgeons at either Nottingham or Leicester. Retroperitoneal sarcomas are often large and infiltrative. The goal of “wide excision” is unlikely to be achievable in most cases. The objective should be “planned marginal excision” achieving appropriate margins that balance tumour control whilst minimising operative morbidity and retaining function. However, multivisceral resection may be appropriate o permit en bloc resection of the tumour. Depending on the position of the tumour the expertise of other subspeciality surgeons may be desirable. Local recurrence even after extensive surgery common. The role for post operative radiotherapy is not defined in retroperitoneal sarcomas. It may be useful in individual cases but is not routine practice. Normal tissue tolerances prevent doses above 45 – 50Gy being used and it is difficult to define the radiation volume. In cases where high risk margins can be identified the post operative radiotherapy should be considered. Preoperative radiotherapy is likely to become a treatment option as the treatment volume is smaller and the tumour acts as a natural spacer. International trials are in set up and these should be supported. New radiation techniques such as IMRT are likely to make radiotherapy in retroperitoneal sarcomas a more beneficial treatment. There is currently no evidence to support the use of neo adjuvant or adjuvant chemotherapy in retroperitoneal sarcomas. Unresectable or metastatic Systemic chemotherapy delivered as described for limb/trunk. If clear response on imaging reconsider surgery. Inoperable disease post-chemotherapy should be considered for consolidation radiotherapy. The dose delivered depends on the extent of the disease, PS of the patient and their likely survival. Pelvic Resectable non-metastatic As per retroperitoneal sarcomas. If post operative radiotherapy is likely to be needed a spacer should be inserted at the time of surgery. Supraclavicular Resectable non-metastatic EMCN Sarcoma Management Guidelines – Updates 30 11 11 35 Tumour resection should be undertaken by the appropriate site specific team after discussion with the sarcoma MDT. There is no role for routine neck nodal dissection. There is no role for neo-adjuvant therapy but post-operative radiotherapy should be considered after review within the sarcoma MDT. Resection margins are expected to be narrow and/or involved. Standard RT planning margins are not likely to be attainable but due care in considering tissue planes that have not been compromised should be employed. RT doses as for trunk/limb. Technical RT delivery should be undertaken by the appropriate site specific team. Unresectable or metatstatic Unresectable but non-metastatic disease should be treated with primary RT using pseudoradical doses.Neo-adjuvant combination chemotherapy may be considered for synovial sarcoma and myxoid liposarcoma as described in limb/tunk. Neo-adjuvant therapy is not otherwise recommended. Metastatic disease should be managed as described in limb/trunk. If unfit for chemotherapy and minimal systemic disease (ie locally progressive disease will/may be unacceptably morbid within the patient’s expected life-span) then consider surgical resection and/or high dose RT for local control. Soft tissue tumours of vascular origin Angiosarcoma Operable non-metastatic Often arises on a background of soft-tissue field change. Wide excision with generous margins is indicated followed by wide-field adjuvant RT. RT constraints may mean that radical surgery (amputation) is required. Unresectable or metastatic Systemic therapy is indicated. Photography of index lesion (with measurement) may be helpful for cutaneous disease. Palliative radiotherapy as described for limb/trunk. Doses may be limited by prior RT. Pulmonary metastases are commonly sub-pleural and cystic with a higher than average risk of spontaneous pneumothorax. Consider intervention (VATS pleurodesis or palliative RT) for such lesions. First-line Doxorubicin. Second line Weekly paclitaxel 90mg/m2 for up to 24 weeks Kaposi’s sarcoma Under the remit of cutaneous lymphoma MDT Paediatric-type Ewing’s sarcoma All patients should be considered for EURO-EWING99. Non-randomised patients should be managed according to the control arm of this trial. Stage specific modality scheduling with respect to systemic therapy EMCN Sarcoma Management Guidelines – Updates 30 11 11 36 Localised tumours Delayed RT possible Induction: VIDE x 6 with tumour evaluation after every 2nd cycle Resection: Should follow induction chemotherapy unless emergency (eg spinal cord compression). If intra-lesional/marginal resection anticipated and/or progression on induction consider pre-operative RT. If inoperable consider primary RT. Consolidation: VAI x 8 with radiotherapy (see below) Delayed RT not possible Induction: VIDE x 6 with tumour evaluation after every 2nd cycle and RT concurrent with cycles 5 & 6 (omit doxorubicin) Resection: see above Consolidation: VAI x 8 Metastatic disease Pulmonary/pleural Induction: VIDE x 6 with tumour evaluation after every 2nd cycle Resection: See above Consolidation: VAI x 8, radiotherapy, pulmonary metastasectomy, whole lung RT Extra-pulmonary Induction: VIDE x 6 with tumour evaluation after every 2nd cycle Resection: see above Consolidation: Best supportive care or high-dose chemotherapy with radiotherapy to primary and metastatic sites Schedule specific radiotherapy timing (for doses see EURO-EWING99 protocol) Pre-operative RT Give concurrent with cycles 5 & 6 VIDE. Primary RT Give following 6 VIDE or 8-10 weeks post high-dose engraftment. Post-operative RT Give concurrently following cycle 7 (ie with cycle 2 VAI and subsequent cycle(s)). Indicated for marginal and intralesional resections and where ≥10% viable cells histologically. Whole-lung RT To follow completion of VAI x 8 Other metastases See EURO-EWING99 protocol Second line chemotherapy Irinotecan and temozolamide Rhabdomyosarcoma Patients with rhabdomyosarcoma, excluding pleomorphic rhabdomyosarcoma (adult-type) should be considered for EPSSG RMS2005. Non-randomised patients should be managed as per the control arm of this study. Full guidance is contained within the EPSSG protocol. Risk grouping is set out in the table. Treatment by risk group is available from the trial protocol EMCN Sarcoma Management Guidelines – Updates 30 11 11 37 Borderline/non-malignant soft tissue tumours Fibromatosis / Desmoid tumour Fibromatosis is a benign, clonal tumour with can be locally aggressive although does not metastasise. It can occur in association with familial adenomatous polyposis when it is called Gardner’s syndrome. A family history must be taken in all cases and if any suggestion of FAP then a referral to the genetics team should be offered to the patient. Intra-Abdominal Tumours Resectable and symptomatic o Resect Resectable asymptomatic o Observe whilst stable disease (see non-progression data), or if o Slow progressive disease continue to observe or tamoxifen + NSAID until stabilisation plus 6 months after, then stop, or if o Rapidly progressive resect if still resectable. Un-Resectable o If symptomatic chemotherapy or radiotherapyMethotrexate and Vinorelbine Radiotherapy depending on the radiation volume. Aim to give 54Gy in 1.8 Gy fractions. Small bowel tolerance may limit this dose. o Asymptomatic observe, or if o Slow progressive disease tamoxifen and NSAIDs, or if o Rapid progressive disease methotrexate and vinorelbine or radiotherapy. Extra-abdominal and abdominal wall (tend to more aggressive intervention with H&N site) Resectable and symptomatic o Resect o Indications for adjuvant radiotherapy: For a first resection: positive margins, and a risk there is a subsequent local recurrence would be inoperable. For after resection of a local recurrence: a positive margin or a negative margin with a risk that subsequent recurrences would be inoperable. We would not usually use adjuvant radiotherapy for second recurrent tumours that might be resectable at a later recurrence. Adjuvant dose of radiotherapy should be 50.4Gy in 1.8 Gy fractions. If macroscopic disease 54 Gy in 1.8Gy fractions. Large margins i.e. 5 cm are necessary. • Resectable and asymptomatic • Observe while stable disease, or if • Slow progressive disease either continue to observe or use hormones and a NSAID or if • Rapid progressive disease resect or hormones, or if • Fast progression resect or chemotherapy Unresectable and asymptomatic o Observe, or if o Slow progressive disease? hormones and NSAID, or if o Rapid progressive disease use chemotherapy and radiotherapy, or if EMCN Sarcoma Management Guidelines – Updates 30 11 11 38 o Painful with a borderline resectability primary chemotherapy and if they respond then observe, if progress then radiotherapy. Unresectable and symptomatic o Chemotherapy or radiotherapy (local morbidity) Role of genotyping 45F beta-catenin gene mutation carriers had a 77% odds of recurrence at 5 years (95% CI 90% to 60%). They had a 90% 10-year recurrence rate. This was about 40% of cases. 41F or no mutation carriers (60% of the cases) had a 43% odds of recurrence at 5 years (95% CI 31-57%). Therefore for the 45F patients we would seek to avoid resection, and either observe or use medical therapies as the primary management. IHC for beta catenin can be indicative of risk, but this achieved much less good definition of risk of recurrence cases. If we had the antibody we could collect that data as well as genotyping while we were piloting this approach. ER beta IHC staining might be useful in that there might be a correlation between staining and response to hormonal treatment. Medical therapy Endocrine: Tamoxifen 40 - 60 mg with Diclofenac 50mg TDS Cytotoxic: Vinorelbine/methotrexate Liposomal doxorubicin is currently not agreed for funding by the commissioners but can be applied for on an individual patient basis Dermato-fibrosarcoma protruberans DFSP is a rare neoplasm of the dermis layer of the skin. It can be considered as a borderline malignancy that rarely metastasises but can be locally aggressive. Wide excision is essential except where it may result in significant morbidity or functional loss. Mohs surgery should be considered. Radiotherapy should be considered if surgery is not possible In unresectable or metastatic disease treat with chemotherapy as for soft tissue sarcomas. DFSP is driven by t(17;22) that results in the over expression of PDGF. Imatinib is licensed for the treatment of unresectable DFSP and if needed funding will need to be sort on an individual basis from the commissioners. Soft Tissue Tumour of Uncertain Malignant Potential (STUMP) Manage as for low grade sarcoma. Adjuvant RT not proven. RT not usually indicated unless surgical options on relapse excessively difficult. Lipomata Symptomatic or lipomata > 5 cm should be marginally excised. Adjuvant therapy not indicated. All resected lipomata should be submitted for both histopathological and cytogenetic analysis. Malignant bone tumours Bone tumours fall outside the remit of the East Midlands (EMCN) Sarcoma Group. All suspected primary tumours of bone should be referred to a recognised Bone Sarcoma MDT. EMCN Sarcoma Management Guidelines – Updates 30 11 11 39 Current practice is to refer cases to the Royal Orthopaedic Hospital in Birmingham or the Royal National Orthopaedic Hospital, Stanmore where a comprehensive rapid access staging, biopsy and surgical service operates. It is recognised that there are links with the Glasgow Bone Tumour Service for bone tumours arising within the facial skeleton and baseof-skull. The East Midlands Sarcoma Group CANNOT provide recommendations for a definitive overall management plan for bone sarcomas as this is the role of the specialist Bone Sarcoma MDTs but it may participate in delivering integrated non-surgical aspects of treatment. Osteosarcoma Patients should be considered for randomisation into either EURAMOS (age <40 years) or EUROBOSS trials. Non-trial patients should be managed as per the control arms of these trials. Further information is available from the trial protocols. Induction therapy: Cisplatin, doxorubicin and methotrexate x 2 Surgery to primary disease: Surgery to metastatic disease (after cycle 2 and before cycle 5) Consolidation therapy: Cisplatin, doxorubicin and methotrexate x 4 Radiotherapy: Inadequate margins or inoperable disease (R1: 60Gy, R2: 66Gy) Inoperable: 70Gy (from EUROBOSS1) Second line chemotherapy Ifosfamide and doxorubicin and methotrexate(if not received on the Euramos trial). If already received ifosfamide and etoposide then treat with gemcitabine and docetaxel. MFH of bone Manage as for osteosarcoma Ewing’s sarcoma of bone Manage as for Ewing’s sarcoma of soft tissue Chondrosarcoma De-differentiated chondrosarcoma Manage as for osteosarcoma Mesenchymal chondrosarcoma Manage as for Ewing’s sarcoma Chondrosarcoma NOS Localised No role for systemic therapy. Primary surgical excision. No routine role for adjuvant radiotherapy, high doses in selected cases. Metastatic Manage as for adult-type soft tissue sarcoma. Chordoma Multi-modality combined surgical and radiotherapeutic approach using appropriate implants and photon/proton therapy required. Specific advice on the need for, timing and nature of (neo-) adjuvant treatment(s) should be sought from the over-seeing Bone Sarcoma MDT. Information from the Bone Sarcoma MDT should be timely, specific and explicit in determining the roles of the East Midlands Sarcoma EMCN Sarcoma Management Guidelines – Updates 30 11 11 40 MDT non-surgical oncology (NSO) members as they are accountable to the Bone Sarcoma MDT. The same applies to surgical procedures undertaken by EMCN-based surgeons on behalf of the Bone Sarcoma MDT. 10.0 Palliative Care Within EMSS, joint working with local palliative care teams is an important aspect of the service in meeting the needs of patients when they require palliative care. Unfortunately not all patients with sarcomas are curable, and therefore referrals are made as early as possible in order to offer the best palliative and supportive care to the patient and their families. Palliative care is defined by the World Health Organisation as ‘an approach that improves the quality of life of patients and their families facing the problems associated with life threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other symptoms, physical, psychosocial and spiritual’. Palliative care is generally offered by most medical and nursing staff in hospitals and community settings, and can help with basic symptom control as well as psychological, social and spiritual concerns. However, specialist palliative care services are focused on dealing with more complex issues. They are able to give a more of specialist service, consisting of palliative care physicians, clinical nurse specialists and other professionals who work closely with the sarcoma team. Involvement of specialist services may be appropriate at various stages of the illness and treatment, including the early stage, whilst patients are undergoing disease modifying treatment. If they have significant symptom control issues e.g. severe uncontrollable pain, the specialist palliative care team can provide advice and support until the pain is controlled. They will then get involved again if the need arises. This service can be offered in hospitalsinpatient and outpatient settings, patients’ homes, hospices, and the team can visit nursing and residential homes. In addition, some patients benefit from an inpatient admission to a hospice / specialist palliative care unit for symptom control, rehabilitation or terminal care. A multi-professional approach is key to high quality specialist palliative care, with involvement of specialist clinicians, nurses, physiotherapists, occupational therapists, social work, religious/spiritual care and other allied health professionals. Also, there is access to family support services, respite care, complimentary therapies, Marie Curie services, hospice at home and bereavement care. MDT’s meetings are held on a regular basis to discuss individual patient issues i.e. planning of treatment, preferred place care, end of life concerns and complex discharge planning. Information on the above services delivered locally within EMSS and nationally can be obtained on local hospital websites and at Macmillan cancer information centres: Macmillan Cancer Information Centre Leicester Tel: 0116 258 6189 email: [email protected] Website: www.uhl-tr.nhs.uk/cancerinfo Macmillan (cancerbackup) Information Centre Nottingham Tel: 0115 840 2650 Cancer & Palliative Care Information Centre Derby EMCN Sarcoma Management Guidelines – Updates 30 11 11 41 Tel: 01332 254 904 Macmillan Cancer Information & Support Centre Northampton Tel: 01604 544 211 Macmillan Cancer Information & Support Centre Lincoln Tel: 01522 573799 11.0 Rehabilitation Surgery for sarcoma, especially sarcoma of the limbs, can result in significant early and late functional deficits. Early deficits may be overcome to be replaced by later evolving side effects of RT or compensatory changes. Appropriate assessment and input by the MDT should aim to minimize the impact of these changes. Physiotherapy EMSS has a dedicated sarcoma physiotherapist. Their opinion should be sought on all cases of sarcoma discussed by the MDT, though it is not anticipated that formal physiotherapy assessment and input be required for all cases. General goals To provide a seamless, patient-centred physiotherapy service for adults with bone and soft tissue sarcoma that can be accessed from initial contact and at any stage during their treatment pathway. Role of the Sarcoma Physiotherapist • To work as a core member of the EMSS Soft Tissue Sarcoma team, providing soft tissue and bone sarcoma patients (as in- and out-patients) with Specialist physiotherapy input from diagnosis and along their treatment pathway including surgery, radiotherapy, chemotherapy and during palliative treatment. • To ensure that each sarcoma patient is able to access physiotherapy that is specific to their individual needs at the optimum time and provided by the most appropriate physiotherapist. This process involves a combination of both direct physiotherapy input and appropriate colleague liason. • Integrated role summarised in attached chart Outcome measures The sarcoma physiotherapist will take a lead in performing serial functional assessments and recording TESS (Toronto Extremety Salvage Score) outcomes. Specialist Rehabilitation A non-exhaustive list of services includes prosthetics and orthotics, lymphoedema services, dietetics and speech and language therapy. Access will be tailored to the patient’s needs and should be delivered as close to the patient’s home as possible as limited by the geographic availability of specialist support. EMCN Sarcoma Management Guidelines – Updates 30 11 11 42 12.0 Patient information & support Sarcoma is a rare disease and general awareness in its regard is limited. Diagnostic, treatment, rehabilitation and follow-up pathways are also complex and cross many multiprofession, specialty and geographic boundaries. Provision of reliable information is essential and should be provided to the patient, family/carers and other healthcare professionals in an appropriate format and timescale that meet the individual’s needs. Whilst every core-member of the EMSS MDT has a responsibility to provide clear verbal and written communication at each stage of the patient pathway, the Sarcoma Clinical Nurse Specialist (CNS) has a particularly important bridging/continuity and reinforcement role. The Sarcoma CNS The Sarcoma CNS is responsible for the management of a defined caseload of patients with Sarcoma, providing expert nursing advice and support to those patients with Sarcoma and other health professionals in relation to this patient group. They carry continuing responsibility for the assessment of care needs, the development, implementation and evaluation of programmes of care and the setting of standards of care. Role of the Sarcoma Clinical Nurse Specialist To be present at the point the patient has first contact with the core EMSS team. This may be during the diagnostic phase when there is a suspicion of sarcoma and the patient is undergoing investigation. It is not possible for the sarcoma CNS to be present at all imaging (prediagnostic) appointments, though telephone contact will be made when feasible. It is therefore essential that the referring team provide accurate information about the diagnostic process to the patient and that they supply appropriate EMSS literature. To act as the patient’s Key Worker and ensure contact details are provided to the patient and carer. To offer support and provide information (Patient Information Pathway). To direct the patient to where further information, advice and support is available. The CNS will perform a holistic assessment and develop a care plan to meet the needs identified in the assessment. They will coordinate the patient’s movement through their care pathway. For those patients undergoing surgery, the Sarcoma CNS will provide support and information in the pre and post operative period. They will visit the patient whilst an in-patient and will liaise directly with the ward health care professionals ensuring follow up arrangements are in lace. The CNS will be present at the other key discussion points (Sarcoma Pathway) that take place between the patient and medical staff regarding transitions between phases in the patient pathway: adjuvant therapy, suspected relapse, palliative and end-of-life care. The Sarcoma CNS will provide support and relevant information at those points and ensure that relevant contact details are still available. They will visit those patients who require in-patient treatment and will again liaise directly with the ward health care professionals to ensure continued coordination of patients care. EMCN Sarcoma Management Guidelines – Updates 30 11 11 43 They will support the patient and their families at those times ensuring they receive the required information to enable them to participate in their care delivery. They will liaise with Community Services, to ensure seamless provision of care delivery. Those Community Services will include District Nurses, Macmillan Teams and Hospices. The ‘information prescription’ Written patient and carer information is required for each step in the patient pathway. This does not replace face-to-face verbal communication and patients should also be offered copies of clinic letters if they wish. Patients who choose to receive copies of clinic letters must understand that the letters are primarily for communication between medical professionals but that they may help their recall of issues discussed during consultations and of plans agreed. Clinicians should realise that information of a potentially damaging nature (suspicions of relapse or prognostic estimates) contained in these letters should have been discussed with the patient. Information checkpoints Written information for use in the following circumstances is available or is in development. The wide spectrum of STS is such that both generic and specific information may be required, dependant upon circumstances. Suspected diagnosis Imaging Biopsy Diagnosis Malignant Benign Treatment Surgery Radiotherapy Chemotherapy Rehabilitation Follow up Relapse Palliative care End-of-life care Additional resources MacMillan Cancer Support SarcomaUK GistUK EMCN Sarcoma Management Guidelines – Updates 30 11 11 44 13.0 Follow up (Demonstrating Compliance with Measure 11-1C-111l, 11-1C-112l) Soft Tissue Sarcoma Referred to the Sarcoma Service Following completion of an episode of active treatment, from and including the referral to whoever is undertaking follow up. It recognises that some sarcomas with site-specific presentations will be managed according to the agreed area shared care pathways. It covers follow up until this ends and includes the process of referral back to the MDT when recurrence is diagnosed by the follow up clinic. Follow-up for treated sarcoma patients incorporates scheduled screening for local and systemic relapse, for the early and late consequences of local and systemic therapies and the provision of psychological support. No study has clearly shown an improvement in overall outcomes as a consequence of routine follow-up, though most agencies recommend follow-up as a component of survivorship care. A careful balance must be struck between opportunity gain arising from follow-up and the patient anxiety potentially provoked by it. As symptomatic relapse may occur between scheduled follow-up appointments it is essential that patients are informed of how to make contact with the sarcoma service so that unscheduled review can be arranged. Components of scheduled follow-up care Clinical assessment Relapse and toxicity screening enquiry (local, end-organ and systemic) and physical examination. Toxicity recording ideally with CTCv3/4 Imaging Local: There is no role for baseline post-treatment local imaging unless either (a) abdomino-pelvic primary or (b) non-abdomino-pelvic site but difficult to follow clinically. Clinical circumstance will dictate modality, notionally CT for abdomen/pelvis and MRI for other sites. Systemic: Chest x-ray. Laboratory tests There is no specific marker for sarcoma relapse at this time. Routine haematology, biochemistry (U&E, LFT, Bone) is indicated if: (a) patient has received systemic therapy. (b) patient has had radiotherapy with potential effect upon end-organ function (essentially any non-limb site). (c) primary tumour was abdomino-pelvic Specific tests of end-organ function TESS score should be completed for limb sarcomas. A post-treatment echocardiogram should be performed 3 months after completion of any adjuvant anthracycline containing regimen. Formal pulmonary function tests (including DLCO) should be performed 12 months after whole-lung radiotherapy. An endocrine profile is required if post-treatment endocrinopathy is possible. EMCN Sarcoma Management Guidelines – Updates 30 11 11 45 Procedure on suspected relapse Patients with suspected relapse of the basis of either history and physical findings or screening investigation should be re-imaged with the definitive imaging method (U/S and MRI or CT for local relapse and CT for systemic relapse). If equivocal this imaging should be re-discussed at the Sarcoma MDT meeting. Confirmatory biopsy may be required. Follow-up post-relapse Patients follow-up should be ‘re-zeroed’ at the time of completion of relapse management and they should enter follow-up based upon the disease risk group as defined above. Follow-up for patients who have exhausted effective anti-cancer treatment There may be no practical benefit to patients in travelling into the specialist sarcoma clinic if there is no possibility of sarcoma specific intervention. Adequate generic supportive and palliative measures may be delivered at a community and local district hospital level. Routine appointments are not usually necessary provided adequate lines of communication and responsibility have been established closer to the patient’s home. It is recognised that sarcoma is a rare disease and that this may provoke some anxiety in the locally based palliative care teams. It is essential that a central route of access for information provision and discussion remain open and that this is clearly communicated. Scheduling of routine appointments, if still felt to be necessary, should be based upon the patient’s individual needs and anticipated disease trajectory. EMSS Follow-Up Strategy for Soft Tissue Sarcomas Stage of Disease Year Disease Monitoring Localised Extremity Tumour treated by surgery +/- radiotherapy ALT Year 1 – 2 GRADE I TUMOURS $ (Excluding ALT) Baseline Year 1 Year 2 Years 3+ GRADE II AND III Baseline EMCN Sarcoma Management Guidelines – Updates 30 11 11 3 monthly Clinical Examination for year 1 and 6 monthly for year 2. Consider MRI 3/12 post-surgery for large, deep, impalpable tumours as baseline Surveillance MRI as clinically indicated. CT Chest at diagnosis 6/52 post-operative check 3 monthly Clinical Examination & CXR MRI 3/12 post-surgery for impalpable tumours as baseline 6 monthly Clinical Examination & CXR Annual Clinical Examination & CXR for minimum of 10 years CT Chest at diagnosis 46 TUMOURS $ Year 1 Year 2* Years 3-5* Years 610* 6/52 post-operative check or 4/52 post-radiotherapy check 3 monthly Clinical Examination, CXR Baseline MRI 3 months after completion of treatment 3 monthly clinical examination & CXR 6 monthly clinical examination & CXR Annual clinical examination & CXR $ Surveillance MRI in addition to other surveillance where indicated: eg. Tumours at high risk of local recurrence, Tumour sites difficult to assess clinically and where surgical margins are compromised by anatomy (neurovascular bundle etc) * Where treatment has been multi-modality, appointments should be alternated between Sarcoma Surgeon and Sarcoma Oncologist EMSS Follow-Up Strategy for retroperitoneal Sarcomas Abdominal, Retroperitoneal & Gynaecological Sarcomas post-surgery GRADE I Year 1-2 Year 3-10 GRADE II & III Year 1 Year 2 - 4 Year 5 - 10 6 monthly clinical examination Annual CXR CT chest/abdo/pelvis if clinically indicated Annual clinical examination & CXR CT chest/abdo/pelvis if clinically indicated 3 monthly clinical examination and CXR Baseline CT chest/abdo/pelvis at 3 months post-surgery then at 12 months 6 monthly clinical examination and CXR CT chest/abdo/pelvis at 24 & 36 months Annual clinical examination & CXR CT chest/abdo/pelvis if clinically indicated The Follow up Pathway for Bone Sarcoma (Demonstrating Compliance with Measure 11-1C-112l) The Follow Up Pathway for Bone Sarcoma is included in Appendix 1. EMCN Sarcoma Management Guidelines – Updates 30 11 11 47 EMCN Soft Tissue Sarcoma Pathway Appendix 1 EMCN Sarcoma Management Guidelines – Updates 30 11 11 48 Key Acute Key Worker Primary Holistic Assessment Both Bone Sarcoma Pathway Patient Information Patients Journey From Home To Home 14 Days Patient Pathway Timeline Referrals: - GP - Radiology - Surgery - Pathology (Northampton & Kettering Hospitals) Referrals: GP Radiology Surgery Pathology (Lincoln, Mansfield & Burton Hospitals) Patient should be fully staged by Day 31 Referred to Sarcoma Clinic in Leicester East Midlands Sarcoma MDT Referred to Nottingham 31 Days Referred to Oxford or Stanmore or Birmingham for Surgery Diagnosis/ staging & Treatment plan agreed 62 Days Chemotherapy under direction of sarcoma oncologist in Leicester Radiotherapy under direction of sarcoma clinical oncologist in Leicester or Northampton Chemotherapy under direction of sarcoma oncologist & Radiotherapy under direction of sarcoma clinical oncologist in Nottingham Follow up Follow up shared between bone and local centre under direction of bone centre Treatment Complete End of Treatment Results Discussed Remission Long-term follow-up Survivorship Relapse Discussion at MDT Palliative Care End of Life provided locally Treatment Options: Chemotherapy, Radiotherapy and Surgery Palliative Care End of Life Locally Remission Long-term follow/up Survivorship Reviewed November 2011 EMCN Sarcoma Management Guidelines – Updates 30 11 11 49 Key Acute Key Worker Primary Holistic Assessment Both Soft Tissue Sarcoma Pathway Patient Information 18/19 – 24 years Patient Pathway Timeline 14 Days 31 Days 62 Days Follow up Support for patient – specialist Nurses, Psychologists, Clic Sergeant, Youth Workers, Education, Nutritionist and Fertility Services Referral from GP Patients should be fully staged by Day 31 Referred to Sarcoma Outpatient Clinic Excision biopsy performed at Nottingham or Leicester Referrals: Radiology Surgery Pathology Patient seen locally by Sarcoma clinician to assess and explain next steps Diagnosis and Treatment plan agreed East Midlands Sarcoma MDT and TYA MDT Surgery performed at Nottingham Follow up after first line management jointly in Leicester / Nottingham and Local Area Chemotherapy under direction of sarcoma oncologist Palliative Chemotherapy can be given locally Radiotherapy can be given locally under direction of sarcoma clinical oncologist in Nottingham or Leicester Palliative Radiotherapy locally Treatment Complete End of Treatment Results Discussed Remission Long-term follow-up Survivorship Relapse Discussion at Sarcoma MDT & TYA MDT Palliative Care End of Life provided locally Treatment Options: Chemotherapy, Radiotherapy and Surgery Palliative Care End of Life Locally Remission Long-term follow/up Survivorship Reviewed November 2011 EMCN Sarcoma Management Guidelines – Updates 30 11 11 50 Key Acute Key Worker Primary Holistic Assessment Both Bone Sarcoma Pathway Patients Journey From Home To Home 18-24 yr olds Patient Information 14 Days Patient Pathway Timeline Referrals: - GP - Radiology - Surgery - Pathology (Northampton & Kettering Hospitals) Referrals: GP Radiology Surgery Pathology (Lincoln, Mansfield & Burton Hospitals) Referred to Sarcoma Clinic in Leicester East Midlands Sarcoma MDT Referred to Nottingham 31 Days 62 Days Follow up Patient should be fully staged by Day 31 Chemotherapy in Leicester Follow up shared between bone and local centre under direction of bone centre Referred to Oxford or Stanmore or Birmingham for Surgery Diagnosis/ staging & Treatment plan agreed Radiotherapy in Leicester or Northampton Treatment Complete End of Treatment Results Discussed Remission Long-term follow-up Survivorship Chemotherapy & Radiotherapy in Nottingham Relapse Discussion at MDT Palliative Care End of Life provided locally Treatment Options: Chemotherapy, Radiotherapy and Surgery Palliative Care End of Life Locally Remission Long-term follow/up Survivorship Reviewed November 2011 EMCN Sarcoma Management Guidelines – Updates 30 11 11 51 EMCN Sarcoma Cancer Pathway Details/ Supporting Information a) Referral • • • • • Cutaneous lesions should first follow the EMCN Melanoma/ Non Melanoma Pathways Suspected groin/axilla/neck lymph nodes should follow the EMCN Lymphoma Pathway IOG – Referral may be sent as urgent if: >5cm Deep/fixed Increasing Size Symptomatic Site of previous surgical resection Direct referrals to the MDT may come from pathology or radiology (supporting pt information should ideally be available for discussion at the MDT) Referral criteria to be regularly audited b) Triage/ Diagnosis • Ultrasound to be undertaken in accordance with agreed Network protocols • Diagnostic machine used must be subject to 6 monthly QA for image acquisition and production • Report to include confirmation/ details of MRI and request that patient is referred to MDT • MRI undertaken in accordance with Network agreed protocols. Images to be rapidly sent to SMDT • IOG – required Triple Assessment undertaken at this point • Biopsy to be undertaken by core member of SMDT • Access to on site cytogenetics required c) Treatment • Surgery to be undertaken at agreed Specialist Treatment Centre (City Campus Nottingham University Hospital) • IOG – compliant membership required for SMDT • Radiotherapy and Chemotherapy to be undertaken at agreed designated Centre Surgical Follow-up with MDT surgeon. Shared follow up care with local trust’s surgeon may be appropriate in some cases. EMCN Sarcoma Management Guidelines – Updates 30 11 11 52
