Chapter 73 • MPM is a rare disease with 2,000 to 3,000 cases annually in the United States. • It is associated with prior occupational asbestos exposure, but some patients without known asbestos exposure will develop MPM. • Four subtypes exist: epithelioid, sarcomatous, biphasic epithelial (also referred to as mixed), and desmoplastic. • Epithelioid is the most common and may be associated with a better prognosis. • Diagnosis must be differentiated from metastatic adenocarcinoma or non–small cell lung cancer with adenocarcinoma histology. No single test is diagnostic of MPM, and it frequently requires a panel of immunohistochemistry markers to confirm the diagnosis. • Cytologic analysis of pleural fluid is not reliable to exclude MPM, and many times a thoracoscopic biopsy may be required for definitive diagnosis. Evidence of invasion into subpleural adipose tissue is the most reliable indicator of malignancy. • Computed tomographic (CT) scan is the initial staging procedure. • Positron emission tomography (PET) or PET-CT scan has the ability to detect extrathoracic disease. • Cervical mediastinoscopy may be useful for detecting mediastinal involvement. • Peritoneal lavage or laparoscopy is indicated if peritoneal involvement is suspected. • For patients with operable disease without significant co-morbidities, surgical options include extrapleural pneumonectomy or pleurectomy and decortication. • Single-institution and phase II trials have demonstrated the feasibility of multimodality therapy. • The benefit of post- or preoperative radiation and chemotherapy is undefined. • For patients with unresectable disease or metastatic disease, without significant treatment co-morbidities, and preserved performance status, treatment with a platinum agent (cisplatin or carboplatin) and antifolate (pemetrexed or raltitrexed) is the standard therapy. • A variety of agents have shown activity in the second-line setting.
Diseases of the Pleura and Mediastinum
Summary of Key Points
Malignant Pleural Mesothelioma (MPM)
Epidemiology
Differential Diagnosis
Staging
Primary Therapy
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