Pleural and Chest Wall Tumors

Pleural and Chest Wall Tumors Ritu Gill, MD, MPH Dr. Ritu R. Gill Department t of Radiology Brigham and Women s Hospital Harvard Medical School CLASSIFICATION OF CHEST WALL TUMORS Primary neoplasm of chest wall: malignant, benign Metastatic neoplasms to chest wall: sarcoma, carcinoma Adjacent neoplasms with local invasion: lung, breast, pleura 2 INCIDENCE Primary chest wall tumors are uncommon. 50-80 % of these tumors are malignant. Soft tissues are major sources of chest wall tumors. The most common primary malignant chest wall tumors are malignant fibrous histiocytoma, rhabdomyosarcoma and chondrsarcoma. The most common primary benign chest wall tumors cartilaginous tumors, desmoids and fibrous dysplasia. PRIMARY CHEST WALL TUMORS Malignant myeloma, malignant fibrous histiocytoma, chondrosarcoma, rhbdomyosarcoma, Ewing s sarcoma, liposarcoma, lymphoma, leiomyosarcoma, hemangiosarcoma Benign osteochondroma, chondroma, desmoids, fibrous dysplasia, lipoma, fibroma, neurilemoma 3 4 PRIMARY BONE TUMORS Primary bone neoplasms involving chest wall are uncommon. The most common benign bone tumors are cartilaginous in origin-osteochondroma and chondroma. The most common malignant bone tumors are myeloma, chondrosarcoma, malignant lymphoma and Ewing s sarcoma. BENIGN RIB TUMORS Osteochondroma It is the most common benign bone tumor( 50% of benign rib tumors ) It arises from the metaphyseal region of the rib and present a bony protuberance with a cartilaginous cap. Rarely, it can involves the diaphragm and induce hemothorax. 5 6

BENIGN RIB TUMORS Cystic, non-neoplastic lesion with fibrous replacement of medullary cavity of the rib. Albright s syndrome( multiple bone cysts, skin pigmentation and precocious sex maturity in girls il ) should be suspected if multiple lesions occurs. Treatment t should be conservative. Majority stop growing at puberty. Surgical resection is indicated if pain and enlarging lesions occurs. 7 HISTIOCYTOSIS X Comprises of a spectrum of disease involving the reticuloendothelial system, including eosinophilic granuloma, Letterer-Siwe disease and Hand- Schuller-Christian disease. Histiocytosis X occurs in patients younger than 50 years. Eosinophilic granuloma is limited only bone involvement. Bone lesions may occurs in all types of histiocytosis X and skull is the most common. Surgical resection in solitary disease is curative. Radiation therapy can be helpful in multi-site involvement. 8 MALIGNANT RIB TUMORS It is the most common malignant rib tumor. Most myelomas involving chest wall are systemic myeloma. Most myeloma occurs in people of 40-60 years and rare in people< 30 years. Punched-out lesion with cortical thinning i presents radiographically. Pathologic fracture is common. Local excision is for diagnosis. Radiation is for a solitary lesion and both radiation and chemotherapy for multiple lesion. 5-year survival is 20 %. 9 MALIGNANT RIB TUMORS Chondrosarcoma It is almost a tumor of the anterior chest wall. Most chondrosarcoma occurs in people of 20-30 years and male. All tumors from costal cartilages should be considered d malignancy. Pathological fracture is uncommon. Chest wall chondrosarcoma grows slowly and metastases occur late 10 MALIGNANT RIB TUMORS Ewing s sarcoma 2/3 patients is younger than 20 years. An onion-skin appearance of the surface of the bone may be seen. Pathological fracture is rare. Radiation is the treatment of choice and adjuvant chemotherapy is also used. MALIGNANT RIB TUMORS Osteogenic sarcoma It is more malignant and less common than chondrosarcoma. More common in teenagers and young adults. Men more than women. Serum ALP is frequently elevated. Pathologic fracture is rare. The treatment is wide excision. Radiation is not valuable and chemotherapy is controversial. 5-year survival is 20%. 11 12

MALIGNANT RIB TUMORS TUMORS OF THE MANUBRIUM, STERNUM, SCAPULA AND CLAVICLE Radiation-Associated Malignant Tumors Those tumors are uncommon. Osteosarcoma and soft tissue sarcoma are the most common. The initial radiation is most common for breast cancer and lymphoma. The treatment is similar to tumors arising de novo. Primary tumors of the manubrium and the sternum constitute 15 % of the chest wall tumors, but nearly all are malignant. The sternum is a frequent site metastasis from the breast, thyroid and kidney. The scapula is not a frequent site of metastasis. Pi Primary tumors of fthe clavicle l are uncommon, but t90% are malignant. The clavicle is more a site of a metastasis than a primary tumor. 13 14 BENIGN SOFT TISSUE TUMOR Desmoid 40 % of all demoids occur in the chest wall and the shoulder. Encapsulation of vessels and brachial plexus in arms and neck is common. The tumor may extend into the pleural cavity and displace mediastinal structure. Desmoid is most common in people of between puberty and 40 years of age. Men and women was affected equally. The tumor originates in muscle and fascia. The tumor must be treated with wide excision. Recurrence may occur if inadequate excision. 15 MALIGNANT SOFT TISSUE TUMORS Malignant Fibrous Histiocytoma It is the most common chest wall tumor that the thoracic surgeon was asked. It is most common in people of 50-70 years of age. 2/3 of patients are male. The tumor was unresponsive to radiation and chemotherapy, so wide resection is the choice. 5-year survival is 38 %. 16 MALIGNANT SOFT TISSUE TUMORS Rhabdomyosarcoma It is the most second common malignant soft tissue tumor. It is most common in children and young adults. The tumor is neither pain nor tender, despite rapid growth. Wide excision with postoperative chemotherapy and radiotherapy apy results in 5-year survival of 70%. MALIGNANT SOFT TISSUE TUMORS Liposarcoma It is most common in people of 40-60 years of age. Most patients are men. Treatment is wide excision.5-year survival is 60 %. Chemotherapy and radiotherapy have little to offer. 17 18

MALIGNANT SOFT TISSUE TUMORS PLEURAL MASS CHARACTERISTICS Neurofibrosarcoma Chest wall neurofibrosarcoma occurs along the intercostal nerve. It is most common in people of 20-50 years of age. Most patients are male. Half of patients are associated with von Recklinghausen s disease. Treatment is wide excision. 19 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 p mesothelioma Adenomatoid tumour Lymphoproliferative Disorders Primary effusion lymphoma 9678/3 Pyothorax associated lymphoma Mesenchymal Tumours Epithelioid hemangioendothelioma Angiosarcoma Synovial sarcoma Monophasic Biphasic solitary fibrous tumour Calcifying tumour of the pleura Desmoplastic round cell tumour PLEURAL 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,. Epidemiology of malignant mesothelioma.. May 1, 2009

MALIGNANT PLEURAL MESOTHELIOMA PATHOLOGIC SPECTRUM 3-6:1(men:women) follows exposure Latency of 30-40 years (2000-3000/yr) Present with SOB, chest pain, cough, and weight loss Right side with SVC, Horner syndrome HPO, clubbing A D G B C F I E Epithelioid id 50% Mixed 34% Sarcomatoid 16% H Percent surv rvival 100 80 40 Prognosis of Malignant Mesothelioma is Dependent on the Histologic Type Malignant Mesothelioma Epithelioid Mixed Sarcomatoid 60 WDPM 20 Stage I II III BWH STAGING 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 All of stage I with positive intrathoracic (N1 or N2) lymph nodes and positive resection margins 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) 0 0 24 48 72 96 120 Time (months) IV Distant metastatic disease Sugarbaker, DJ, Norberto, JJ, Swanson, SJ. Surgical staging and work up of patients with diffuse malignant pleural mesothelioma. Mesotheliomas.. 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

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 (white arrows), and extension into the abdomen (arrowhead). IN SOME CASES, COMPLEMENTARY ANATOMICAL INFORMATION CAN BE DERIVED FROM MRI

Accuracy of MPM staging by CT and MRI p T2 T2 Diaphragm involvement Endothoracic fascia involvement or solitary resectable focus tumor MRI IS SUPERIOR TO CT.46.63.69.59.01.05.64 DIFFUSION WEIGHTED IMAGING 3orthogonal directions 4b-values (0,250,500,750) Time 6.5 mts ADC = 0.883x 10-3 mm 2 /s HASTE DW I CE ADC FOLLOWING NEOADJUVANT CHEMOTHERAPY Pre-chemotherapy 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 under- Post-chemotherapy Pre-chemotherapy Prechemotherapy Post-chemotherapy Post-chemotherapy

Course of MPM Pleural effusion dominant early Progressive encasement of lung Lobulated pleural masses late Invasion of lung Lung nodules and distant spread SOLITARY FIBROUS TUMOR Benign mesothelioma Benign fibrous tumor of pleura Localized fibrous tumor of pleura Solitary fibrous tumor SOLITARY FIBROUS TUMOR Rare Symptoms relate to size : cough, chest pain, Cough, dyspnea, chest pain Paraneoplastic syndrome: clubbing, hypertrophic osteo-arthropathy, hypoglycemia SOLITARY FIBROUS TUMOR Visceral pleura 80% Parietal pleura 20% Encapsulated, pedunculated Nodular, whorled appearance CD34 positive; calretinin and WT-1 negative Hemorrhage, necrosis and cysts SOLITARY FIBROUS TUMOR LIPOSARCOMA Typically large, infiltrative, and asymptomatic No evidence to suggest they arise from pre-existing lipomas Heterogeneous masses on CT 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 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

SYNOVIAL SARCOMA Typically occurs in adolescents and young adults between the ages of 15 and 40 years It is believed ed 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 Negative: CD34, S-100 100, GFAP FISH: positive for SYT gene rearrangement LYMPHOMA 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 CONCLUSION Metastases are 95% of pleural lesions Focal pleural tumor can be favorable DX Malignant pleural mesothelioma and Adenocarcinomatosis may look alike RESOURCES Evans AL, Gleeson FV. Radiology in pleural disease: State of the art. Respirology 2004; 9:300-312 Sung SH, Chang JW, Jhingook K, et al. Solitary fibrous tumors of the pleura: surgical outcome and clinical course. Ann Thorac Surg 2005; 79:303-307 Wang ZJ, Reddy GP, Gotway MB, et al. Malignant pleural mesothelioma: evaluation with CT, MR imaging, and PET. Radiographics 2004; 24:105-119119