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Skull Base Tumors Involving the Orbit Donald J. Annino, Jr, MD, DMD a a Skull Base Tumors Challenging to treat Rare Multiple histologies Complex anatomy Orbit – Bony Anatomy Key structure middle 1/3 face 7 bones Communicates with anterior, middle cranial fossae, infratemporal and pterygopalatine fossae Superior and inferior orbital fissures Skull Base Tumors Involving the Orbit Primary Secondary Metastatic Primary Orbital Tumors Benign Inflammatory Vascular – Cavernous hemangiomas Nerve sheath Bony Lymphangioma Primary Malignant Lacrimal gland 50 % malignant ACC, malignant mixed Lacrimal sac Osteosarcoma – after retinoblastoma Rhabdomyosarcoma Secondary Orbital Tumors Paranasal sinuses Intracranial Meningiomas Skin Paranasal Sinus Lesions Benign Osteomas Mucocele Polyps Malignant Carcinoma Sarcoma Mucosal melanoma Paranasal Sinus Tumor ~50 % orbital symptoms Invade by preformed pathways or extension through bone Up to 82 % ethmoid ca invade lamina Up to 50 % invade periorbitum Up to 80 % orbit involvement with maxillary Ganly et al. Head and Neck 2005;27:575-584 Suarez et al. Head and Neck 2004;26:136-144 Metastatic Orbital Tumors 25 % initial presentation Breast Cancer Lung Prostate Melanoma GI tract Renal Cell Neuroblastoma Work-up CT Bone detail MRI Soft tissue Intracranial Ultrasound Ant, MRA middle orbit Work-up Biopsy FNA Open Skull Base Tumors Treatment Sinonasal outcomes improving over 4 decades Improved surgical techniques Conformal radiation Skull Base Tumors Treatment Multimodality therapy Surgery Radiation Chemotherapy Minimize of life morbidity and maximize quality Orbital Tumors Ophthalmologist Head and Neck Surgeons Neurosurgeons Skull Base Tumor Contraindications for surgery Brain involvement Cavernous sinus extension Internal carotid involvement Secondary Orbital Tumors Survival not changed with invasion limited to periorbita Survival impacted with Brain Involvement – dural invasion Histology Orbital soft tissue involvement Suarez et al. Head Neck 2004:26:136-144 Ganly et al. Head Neck 2005:27:575-584 Howard et al. Head Neck 2006:28:867-873 Periorbitum Involvement CT & MRI not accurate Direct observation in OR and frozen section Imola, Schramm. Laryngoscope 2002;112:1357-1365 Indications for Exenteration Extension through the periorbitum Periorbitum not violated but involved then orbit contents preserved Violation of bone alone not indication for exenteration Survival not improved with orbital exenteration/clearance Suarez et al. Head and Neck 2008;30:242-250 Imola, Schramm. Laryngoscope 2002;112:13571365 Surgical Approaches Location Size Goal – Biopsy Debulking Total excision Surgical Approaches Transorbital – Orbitotomy Extraorbital – Endoscopic Anterior Approaches – Lateral Approaches – Subcranial, frontal Frontotemporal, frontoorbitotemporal craniotomy Combined Transorbital Approaches Anterior lesions No extension to the orbital apex Can be combined with extraorbital approaches Transorbital Approaches Anterior with or with out craniotomy Medial Lateral Extraorbital Endoscopic Anterior Skull Base Surgery Approaches Transnasal direct Transseptal Transethmoidal Transsphenoidal Transsphenoidal transclival Extraorbital Approaches Subcranial Raveh Minimizes frontal lobe retraction Excellent exposure to midline and paranasal sinuses Cranialize frontal sinus Extraorbital Approaches Frontotemporal, frontoorbitotemporal Access to orbital apex, superior orbital fissure lesions Enter paranasal sinus pack with fat Intraorbital Dissection Malleable retractors Microscope Microsurgical dissectors Cotton-tipped applicators Extraorbital Approaches Stereotactic navigation Reconstruction Loss of multiple walls – rigid reconstruction Loss floor of floor greater 80% Periorbita reconstructed with fascia, alloderm Orbital roof no reconstruction if alone Pulsations short term Watertight closure of dura Pericranial flap Reconstruction Epiphora - 36 % Silastic stenting – 13% Anderson et al. Otolaryngol Head and Neck Surg 1996;122:1305-1307 Imola, Schramm. Laryngoscope 2002;112:1357-1365 Orbital Tumors DFCI 2006 - 2009 49 patients, 2006- 2009 23 F / 22 M 16 Benign 33 Malignant Orbital 14% (7/49) Secondary 79% (39/49) Paranasal sinus Metastatic 65% (32/49) 6% (3/49) Orbital Tumors DFCI 2006 - 2009 Benign - 16 Mucoceles Cavernous hemangioma Bony -4 -3 -3 Osteoma Fibrous dysplasia Cherubism Meningioma Pseudotumor Schwannoma Malignant – 33 Carcinoma sinuses Sarcoma Esthesioneuroblastoma Mucosal melanoma Metastasis -3 -2 -1 - 12 -5 -4 -3 -3 Breast Thyroid Renal cell Cutaneous Lacrimal sac Lymphoma -4 -2 -1 Presenting Symptoms Diplopia Headache Nasal Obstruction Proptosis 9 9 9 9 Epiphora Decreased visual acuity Epistaxis Anosmia 6 5 4 4 Initial Diagnosis 47 % (23/49) previous procedures 19 Sinusitis 4 Migraine Surgical Approaches Transorbital Subcranial w/ exenteration 2 Frontotemporal Maxillectomy 8 11 w/ exenteration 4 6 16 Endoscopy 8 Surgical Approaches Facial incisions avoided except in transorbital, maxillectomy w/o intracranial extension and exenteration Endoscopic assisted Reconstruction Most tumors involved more than one anatomic area Restore volume Peri-orbitum reconstructed with fascia or alloderm Silastic tubes with orbital preservation and division lacrimal drainage system Temporalis muscle Bone reconstruction Titanium Methyl methacrylate Outcomes 6 patients with intraconal dissection 2 Patients decreased VA (excludes exenteration) 1 Patient improved VA Pseudotumor VA 20/100 to NLP Meningioma VA hand motion no change but decreased visual field Schwannoma 20/300 to 20/70 Diplopia Improved 6 post-op 2 post-op, 1 short term, 1 persists in upward gaze Outcomes Enophthalmos Ptosis 2 patients 3 walls resected 3 patients Epiphora – 0 Lower lid ectropian – 0 Orbital pulsations self-limited CSF – 0 Cerebritis - 1 Case 19 yo M 2 years sx Snoring, nasal obstruction, proptosis Dx – allergies OS – 20/25 OD – able to count fingers Case MRI, CT – Large destructive lesion involving paranasal sinuses, bilat orbits, anterior cranial fossa, middle cranial fossa, extends through clivus Case Endoscopic biopsy Path – Intermediate chondosarcoma Case Subcranial approach Endoscopic assisted Complete gross removal Case No complications Vision unchanged Post-op proton beam Conclusion Tumors involving the orbit require multidisciplinary approach Varied histology Safe, good outcomes with proper planning and approach