Plueral Malignancy: Radiologic-pathologic Correlation Ritu R. Gill, MD Pleural Malignancies: Radiologic-Pathologic Correlation Ritu R Gill MD Brigham and Women s Hospital Boston, Massachusetts Pleural Mass Characteristics Obtuse angles with pleural surfaces Sharp margins on tangential views Discrepant margins on different views Incomplete border May cross fissures Look for osseous and soft tissue--chest wall WHO Histological Classification of Pleural Tumors Mesothelial Tumours Diffuse malignant mesothelioma Epithelioid mesothelioma Sarcomatoid mesothelioma Desmoplastic mesothelioma Biphasic mesothelioma Localized malignant mesothelioma Other Tumours of Mesothelial Origin Well differentiated papillary mesothelioma Adenomatoid tumour Lymphoproliferative Disorders Primary effusion lymphoma 9678/3 Pyothorax associated lymphoma Mesenchymal Tumours Epithelioid id hemangioendothelioma Angiosarcoma Synovial sarcoma Monophasic Biphasic solitary fibrous tumour Calcifying tumour of the pleura Desmoplastic round cell tumour Metastases 40% Bronchogenic adenocarcinoma 20% Breast cancer 10% Lymphoma 30% Other primaries Thymoma Carcinoid Sarcoma Splenosis Malignant Pleural Mesothelioma 2,000-3,000 cases annually in the U.S. Incidence increasing worldwide Up to 10% lifetime risk of developing mesothelioma among asbestos workers Asbestos + cigarette smoking synergistic 60X more likely to develop lung cancer when compared to non-smoking, non-asbestos exposed cohort Sterman, Daniel, et al. Epidemiology of malignant mesothelioma. UptoDate. May 1, 2009 487
Malignant Pleural Mesothelioma Pathologic Spectrum 3-6:1(men:women) follows exposure A D G Latency of 30-40 years (2000-3000/yr) B E H Present with SOB, chest pain, cough, and weight loss C F I Right side with SVC, Horner syndrome HPO, clubbing Epithelioid 50% Mixed 34% Sarcomatoid 16% Prognosis of Malignant Mesothelioma is Dependent on the Histologic Type vival Percent surv Malignant Mesothelioma 100 Epithelioid 80 Mixed Sarcomatoid 60 WDPM 40 20 0 0 24 48 72 96 120 Time (months) Sugarbaker DJ et al, J Thorac Cardiovasc Surg. 1999 Jan;117(1):54-63 Stage I Definition Disease confined to within the capsule of the parietal pleura; ipsilateral pleura, lung, pericardium, diaphragm, or chest wall disease limited to previous biopsy site II All of stage I with positive intrathoracic (N1 or N2) lymph nodes and positive resection margins III IV BWH Staging Local extension of disease into chest wall or mediastinum; heart, or through the diaphragm and peritoneum; with or without extrathoracic or contralateral lymph node involvement (N3) Distant metastatic disease Sugarbaker, DJ, Norberto, JJ, Swanson, SJ. Surgical staging and work up of patients with diffuse malignant pleural mesothelioma. Mesotheliomas. Seminars in Thoracic and Cardiovascular Surgery. Ed. Sugarbaker DJ. Volume 9, issue 4, 1997 Radiographic Presentation Nodular Pleural Thickening Wide range Pleural effusion, small to large Encasement of lung Lobulated pleural masses 488 2
Chest Wall and Mediastinum Involvement Diaphragmatic Extension diaphragm Pulmonary, Lymph Node, and Liver Involvement Chest Wall Invasion Sagittal and coronal MDCT images reveal marked encasement of the right lung with extension of the pleural mass into the mediastinum and extension into the abdomen. In some cases, complementary anatomical information can be derived from MRI 3 489
Accuracy of MPM staging by CT and MRI p T2 T2 Diaphragm involvement Endothoracic fascia involvement or solitary resectable focus tumor.01.05 MRI is superior to CT Diffusion Weighted Imaging 3orthogonal directions 4b-values (0,250,500,750) Time 6.5 mts ADC = 0.883x 10-3 mm 2 /s HASTE CE DW I ADC 490 4
Following Neoadjuvant Chemotherapy Course of MPM Pleural effusion dominant early Progressive encasement of lung Pre-chemotherapy Post-chemotherapy Prechemotherapy Post-chemotherapy Lobulated pleural masses late Rotating maximum intensity projection and fusion PET-PT images of the same patient from pre and post chemotherapy studies show reduced post treatment FDG avidity of the right pleural mass. The patient subsequently underwent successful EPP. Pre-chemotherapy Post-chemotherapy Invasion of lung Lung nodules and distant spread Benign mesothelioma Benign fibrous tumor of pleura Localized fibrous tumor of pleura Solitary fibrous tumor Rare Symptoms relate to size: cough, chest pain, dyspnea Paraneoplastic syndrome: clubbing, hypertrophic osteo-arthropathy, hypoglycemia Visceral pleura 80% Parietal pleura 20% Encapsulated, pedunculated Nodular, whorled appearance CD34 positive; calretinin and WT-1 negative Hemorrhage, necrosis and cysts 5 491
CD34 Calretinin Liposarcoma Typically large, infiltrative, and asymptomatic No evidence to suggest they arise from pre-existing lipomas oge masses on CT Heterogeneous with soft tissue and fat component. They tend to measure less than 50 HU (mean CT values) on pre and post intravenous contrast enhancement images WT1 On MRI, they tend to have high signal intensity on T2WI, because of myxoid degeneration, while low signal is common and have variable enhancement post gadolinium administration Solitary fibrous tumor of pleura Normal adipose tissue Well-differentiated liposarcoma Synovial Sarcoma Typically occurs in adolescents and young adults between the ages of 15 and 40 years It is believed to originate from primitive pleuripotent mesenchyme capable of synovial differentiation Because of its rarity, it is often mistaken on imaging and histology for sarcomatoid mesothelioma Molecular studies for the X:18 translocation are useful for differentiation Focally positive: EMA, AE1/AE3, Cam5.2 De-differentiated liposarcoma Negative: CD34, S-100, GFAP FISH: positive for SYT gene rearrangement Lymphoma LCA CD20 (B) CD10 CD3 (T) Both primary and secondary forms can involve the pleura. Follicular and B cell lymphoma tend to be more common There can be associated lymphadenopathy, as well as chest wall and marrow involvement. Tend to be low on T1WI and iso to high signal on T2WI Diffuse homogenous enhancement Diffuse large B cell lymphoma 492 6
Conclusion Metastases are 95% of pleural lesions Focal pleural tumor can be favorable DX Malignant pleural mesothelioma and Adenocarcinomatosis may look alike Resources 1. Pelucchi C, Malvezzi M, La Vecchia C, Levi F, Decarli A, Negri E. The mesothelioma epidemic in Western Europe: an update. Br J Cancer 2004 Mar 8; 90(5):1022-4. 2. Miller BH, Rosado-de-Christenson ML, Mason AC, Fleming MV, White CC, Krasna MJ. From the archives of the AFIP Malignant pleural mesothelioma: radiologic-pathologic correlation. RadioGraphics 1996; 16:613 644. 3. Kucuksu N, Thomas W, Ezdinli EZ. Chemotherapy of malignant diffuse mesothelioma. Cancer 1976; 37:1265 1274. 4. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981; 47:2678 2689. 5. Suter M, Gebhard S, Boumghar M, Peloponisios N, Genton CY. Localized fibrous tumours of the pleura: 15 new cases and review of the literature. Eur J Cardiothorac Surg 1998; 7 493