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Dr.Waseem HAJJAR MD, FRCS, Assistant Professor & Consultant thoracic surgeon KKUH, King Saud University Solitary Pulmonary Nodule. Benign lung tumors. Carcinoid tumor Carcinoma of the Lung Solitary Pulmonary Nodule Definition. Less than 3 cm. Within the lung parenchyma Presentation: Asymptomatic (incidental finding on chest X ray or CT scan) Significance: To distinguish benign lesion ( tumor – inflammatory ) from early stage highly curable cancer Etiology: Inflammatory granulomas Infection e.g. TB, histoplamosis, coccidiodomycosis Round pneumonia Immune mediated e.g. sarcoidosis, rheumatoid nodules or Wegner granuloma (although usually multiple) Neoplastic Benign Malignant Diagnosis Benign vs Malignant History: Age, smoking, occupation, other primary tumors,…) Imaging: CXR and CT: CXR and CT- Time: if the nodule did not change from a previous radiograph, then it is most likely a benign lesion. CXR and CT- Attenuation: 1. Calcification: Diffuse, central, lamellar or chondroid "popcorn" are patterns of benign lesions. Malignant tumors usually have eccentric calcification. 2. Fat content: indicate benign lesion especially hamartoma. CXR and CT- Margin 1. 2. Speculated margin 90% malignant. Smooth margin: not specific (may be benign or malignant) Vasuclarity (CT with contrast): malignant tumors are highly vascular. PET scan: More than 90% sensitive and specific. Malignant tumors have increased metabolism increase uptake.. Histopathology. Culture. Biopsy: Transbronchial (with fluoroscopy guidance) Percutaneous: core (near), FNA (central) Excisional Bx (open thoractotomy or VATS). Histopathology. Complications: pneumothorax (more than 10%), haemothorax. Treatment 1. 2. Depends on the risk for malignancy. Surgical removal (either open or through VATS) in patients with high risk for malignancy. Observe and follow-up with CT every 3 months for 1–2 years if: There is no radiological change in the size of the nodule for 2 years. There is low risk of cancer. The nodule shows benign appearance. Or if there is a poor operative risk Benign Lung Tumors Rare. Incidental findings as solitary lung nodules. Clinically asymptomatic. Classification: Epithelial: Polyps Papillomas Bronchial mucous gland adenoma Mesodermal: Chondroma, Lipoma, Fibroma (12%), Leiomyoma. Lipoma: Arising from submucosal fat between the cartilaginous rings of the bronchus Presents mostly by endobronchial symptoms Treated by brochoscopic excision Sclerosing haemangioma: Middle-aged women. Solitary nodule partially calcified. Haemangiopericytoma: highly vascular with malignant potential. Benign Lung Tumors Unknown ; Clear cell tumor (sugar tumor) Hamartoma: the most common benign lung tumor. Occur in all ages with slight male predominance. Well circumscribed slow-growing single nodules up to 2 cm in size CT: “popcorn” calcification and fat. Others e.g. plasma cell granuloma, germ cell tumor (teratoma), Xanthoma and inflammatory pseudotumors Definitive diagnosis is made by excisonal biopsy Rigid bronchoscopy diagnostic and therapeutic in case of symptomatic lesions Hamartomas (chondroadenomas) Most common type of benign lung tumor, slow growing lesion Mainly occur in adults. Hamartomas are peripherally located. Grossly, they have a firm marblelike consistency. Histologically, hamartomas generally consist of epithelial tissue and other tissues such as fat and cartilage. Radiologically appears as well-circumscribed single nodules, up to 2cm , it can be localized in the lung CT scanning shows calcification and fat in up to half of the lesions. Parenchymal hamartoma of the lung. The surrounding lung falls away from the well-circumscribed mass, a typical feature of these lesions. The hamartoma shows a variegated yellow and white appearance, which corresponds respectively to fat and cartilage Benign lung tumors can be classified by their origin into: Mesodermal (fibroma, lipoma, leiomyoma, chondroma, granular cell tumor, sclerosing hemangioma) Other (myofibroblastic tumor, xanthoma, amyloid, mucosaassociated lymphoid tumor). Benign Lung Lesion Unknown (hamartoma, clear cell, teratoma), epithelial (papilloma, polyps), NOTE:Adenoma and hamartomas constitute the largest group ofbenign lung lesion Bronchial Carcinoid Used to be called “bronchial adenoma” 5% of all lung cancers. Neuroendocrine (APUD) tumor that arise from Kulchitsky cells. Types of Carcinoid tumor Typical lung carcinoid carcinoid Atypical lung Bronchial Carcinoid Typical Carcinoid: 90% of bronchial carcinoids. Well differentiated, locally invasive tumor with low malignant potential. Rarely cause carcinoid syndrome. Highly vascular. Treated by surgical resection with intraoperative frozen section. Prognosis after resection is excellent. Atypical Carcinoid: Increased cellularity, pleomorphism and mitotic activity. More aggressive with high tendency to metastasize to regional lymph nodes. Surgical excision is the treatment of choice. Carciniod Tumor Carcinoid tumors are a slow-growing cancer that can arise in several places throughout your body. Carcinoid tumors, which are one subset of tumors called neuroendocrine tumors,known as the usually appear in the gastrointestinal tract (appendix, stomach, small intestine, colon, rectum) and in the lungs. Lung Cancer Epidemiology In the past 3 decades, The incidence of lung cancer has increased significantly in developed countries. It is the most common cancer in males and second most common cancer in females. 25% of all cancer death in US, The number one cause of cancer death in both sex. Lung Cancer Etiology Smoking (responsible of 85% of all lung cancers ): type duration amount passive (3% of patients with lung cancer) Environmental: radon gas, asbestos, chromium, silica, arsenic, ionizing radiation, and viral infection Lung Cancer Etiology Dietary: fat and cholesterol vs. fruit , vegetable and vitamin A Preexisting lung disease: COPD , diffuse pulmonary fibrosis and scaring from TB or infarction. Lung Cancer Etiology Inheritance: positive family history has been described as a rare autosomal co-dominant resulting in earlier age of onset lung cancer. Molecular genetics alteration: oncogenes (RAS) or tumor suppressor gene (p53 and RB ) on cell growth. Lung Cancer Classification Based on histological differentiation: 1. • • • 2. • • 3. • • • Adenocarcinoma (45%): The most common type. Tend to be in peripheral lung parenchyma and in alveolar septa Lymph node metastasis is common Squamous cell (epidermoid) carcinoma (30%): Tend to be centrally (major bronchi). Keratinization. Sputum cytology. Invade locally. Large cell (undifferentiated) carcinoma (5-10%) Tend to be in peripheral lung Often cavitate Early metastasis 4. Small cell carcinoma(20%): 5. Carcinoid and uncommon lung cancers. Types of Lung Cancer: The main types of lung cancer are small cell lung carcinoma (SCLC ) and non-small cell lung carcinoma (NSCLC ). Lung Cancer Presentation Local invasion of primary tumor: 70% of the patients are symptomatic. Centrally: Cough dyspnoea, unresolved pneumonia, wheeze and hemoptysis. Peripherally: chest wall pain, pleural effusion, tumor cavitation and dyspnea. Presentation Regional spread within thorax and to mediastinum: Pleura (pain, effusion), chest wall(pain), pericardium(effusion), superior vena cava(obstruction), Pancoast tumor(superior sulcus tumor), cervical sympathetic nerves(Horner syndrome), recurrent laryngeal nerve(hoarseness), phrenic nerve (hemediaphram) or esophagus (dysphasia). Lung Cancer Presentation Distant Metastasis Liver, bone, brain or adrenals Paraneoplastic Syndrome these are the distant effects of the tumor (endocrine, neurological, skeletal, hematological, cutanous) unrelated to the metastasis . There is production of one or more active substances (ACTH, ADH, calcitonin, growth hormone, parathyroid hormone) General: anorexia, cachexia and low grade pyrexia SCLC: ACTH , SIADH and Eton-lambert syndrome Squamous cell carcinoma: PTH. Lung Cancer Diagnosis Hx & PE. Sputum cytology: have little role in routine work-up of patient with suspected lung cancer. Tumor Tumor Bronchoscope Tumor lung-cancer-upper-lobe Lung Cancer Diagnosis Chest x-ray: initial diagnostic procedure of choice in suspected patient. It helps in localizing the tumor, presence of mediastinal or hilar lymph node, pleural effusion and lung consolidation. Adenocarcinoma : Well defined peripheral nodule and May have chest wall involvement. Squamous Lung Cancer: central located, associated atelectasis and hilar adenopathy Large cell carcinoma : peripheral mass may cavitate and association with hilar or mediastinal adenopathy . Small cell tumor: bulky mediastinal lymphadenopathy, large central tumors or rarely a small peripheral tumor Pancost tumor : Tumor at the apex of the lung or superior sulcus that may involve : the brachial plexus sympathetic ganglia Vertebral bodies Leading to pain, upper extremity weakness, and Horner’s Syndrome. Horner’s syndrome : Injury to the cervical sympathetic chain: 1. 2. 3. 4. Ptosis Miosis Anhydrosis of ipsilateral face . Endophthalmosis. Lung Cancer Diagnosis CT scan : useful for assessing the size of primary cancer , proximity to adjacent organ and for planning surgical approaches. F.O. Bronchoscopy. MRI: no advantage over CT , it more accurate in assessing spinal cord invasion. Lung Cancer Diagnosis Bronchoscopy : play an essential role in diagnosis , staging and treatment. Percutaneous needle biopsy: fluoroscopic or CT-guided Mediastinoscopy/Mediastinostomy: its improtant for staging. Thoracoscopy : most useful for examining pleural space for seeding of tumor . Staging of NSCLC (Non Small Cell Lung Carcinoma) Why do we need staging ? What TNM stands for ? Staging of NSCLC: T descriptor # T1 <3cm # T2 1.>3cm invading the visceral pleura 2.within a major bronchus >2cm distal to the carina. 3.Associated with partial atelectasis or obstructive pneumonitis . Cont. # T3 1.tumor of any size+direct invasion of non-vital structure 2.within a major bronchus <2cm distal to the carina. 3.Associated with complete atelectasis or obstructive pneumnitis Cont. #T4 1.tumor of any size+direct invasion of vital organs 2.malignant pleural effusion 3.precardial effusion # Tx primary tumor can not be assessed # T0 no evidence of tumor # Tis carcinoma in situ Staging of NSCLS: N descriptor # N0 -- no nodal met. # N1 -- nodes within the ipsilateral lung # N2 -- subcrinal nodes & paratracheal LN ipsilateral to the tumor. # N3 -- nodes in mediastinum contraleteral to lung tumor or in the supraclavicular region # Nx -- regional nodes can not be assessed Staging of NSCLS: M descriptor M0:*NO DISTANT METASTASIS M1:*DISTANT METASTASIS PRESENT NEW INTERNATIONAL REVISED STAGE GROUPING Stage 0 Stage IA Stage IB Stage IIA Stage IIB Stage IIIA Stage IIIB Stage IV TIS T1, NO, MO T2, NO, MO T1, N1, MO T2, N1, MO T3, NO, MO T1-3, N2, MO T3, N1, MO T4, Any N, MO Any T, N3, MO Any T, Any N, M1 Mountain CF. Chest 1997; 111 Surgical Treatment of Lung Cancer (NSCLC) Principles 1.Complete resection of the primary tumor with its intrapulmonary lymphatic's. 2.Intraoperative frozen sections. 3.Intraoperative lymph node staging. Contraindications to Surgical Resection 1.Distant metastasis (M1). 2.Invasion of central mediastinal structures(T4). 3.Malignant pleural effusion (T4). Surgery for NSCLC by stage Stage I-II Surgery is the most effective treatment lobectomy Radiotherapy may be used if patient is inoperable. Stage III-IV: Local (N0): selected patients (alone or with induction therapy). N2: Controversial. Combined modality (Surgery + radiation) N3 (contralateral LN) surgery is absolutely contraindicated. M1 (Distant Metastasis): palliative surgery Surgical options for lung cancer:1.Wedge/Segmental resection 2.Lobectomy/biloectomy 3.Pneumonectomy 4. Video Assisted Thoracosopic Surgery (VATS) 5.Palliative resections (noncurative). Follow up and outcome:H , E & CXR every 3ms for 2years and every 6ms for subsequent 3years Tumor recurrence: first 2-3 years local recurrence: in the same lung or surgical margin regional recurrence: within mediastinal lymph node. systemic recurrence: contralateral lung or outside thorax. There is increased risk of developing second primary tumor of upper areodigestive tract ( i.e. oropharynx , esophagus , lung ) after having the initial lung cancer tumor. prognosis 5-year survival rates, in the United States are as follows: Stage IA - 75% Stage IB - 55% Stage IIA - 50% Stage IIB - 40% Stage IIIA - 10-35% Stage IIIB - 5% (Stage IIIB lesions are non resectable.) Stage IV - Less than 5% overall is 13% because most of the patient diagnosed in stage IV. Chemotherapy Used in the management of NSCLC (Stage III-IV). Combination (multi-agent) chemotherapy is more effective than single-agent chemotherapy. May be used as: 1.before operation to improve the respectability. 2.postoperative adjuvant (Not proven but considered). 3.radiosensitizers in concurrent chemoradiotherapy. 4.inoperable patient. Other treatment choices are : •Chemotherapy, •Radiation therapy (radiotherapy), including radiosurgery (SRS), or targeted therapy. This means less radiation affects nearby healthy tissues. Small cell lung cancer (SCLC):- Small cell lung cancer (SCLC):Oat cancer –20% Epithelial tumor capable of expressing neuroendeocrine amine precursor uptake and decarboxylase (APUD) marker, which can act as neurotransmitters and hormones. (ACTH, ADH) Presentation: 1.aggressive tumor with Early metastasis. 2.Strongly associated with smoking . 3.Symptoms may be related to: # Local tumor growth # Regional lymph node metastases # Distant metastases # Associated with Paraneoplastic Syndrome # Anorexia, Cachexia & low grade pyrexia # ACTH , SIADH Work up:1. Hx & Ex 2. Routine investgations CBC, LFT, …etc. 3. Imaging a.CXR (Tend to be centrally with bilateral mediastinal lymph node involvement) b.CT of chest c.CT of abdomen d.Bone scan e.CT or MRI of brain 4.Bone marrow aspiration Staging of SCLC:Limited disease: confined to ipsilateral lung, hilar, mediastinal, & supraclavicular LN. Extensive disease: contralateral lung or extra thoracic . Very limited disease: confined to the lung Management of SCLC:Primarily treated with chemotherapy Extensive disease: majority of patient combination chemotherapy 5ys survival < 5% Radiotherapy is used for palliation As in brain metastasis Limited disease : 30 % of patient 50% have complete response to therapy 5ys survival 20% Very limited disease : vey rare chemotherapy 5 years survival 60-70-%