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Key Features

Essentials of Diagnosis

  • Unilateral, nonpleuritic chest pain and dyspnea

  • Pleural effusion or pleural thickening or both on chest radiographs

  • Mesothelial cells in pleural fluid or tissue

  • Commonly, a distant (> 20 years earlier) history of exposure to asbestos

General Considerations

  • Primary tumors arising from the mesothelial surfaces of the pleura (80% of cases) or peritoneum (20% of cases)

  • Mesothelioma is almost always malignant (especially, pleural location), with malignant cells on histology; rarely, mesothelioma is benign (usually, abdominal location) with cystic, adenomatoid, fibrous changes on histology

  • 75% of pleural mesotheliomas are diffuse (usually malignant)

  • The average interval between onset of symptoms and diagnosis is 2–3 months, with median age at diagnosis of 72 to 74 years in Western countries

  • The latent period between asbestos exposure and onset of symptoms ranges from 20 to 40 years

Demographics

  • Men outnumber women 3:1

  • Malignant pleural mesothelioma is associated with asbestos exposure (60–80% of cases); the lifetime risk to asbestos workers is 10%

  • Cigarette smoking significantly increases the risk of bronchogenic carcinoma in asbestos workers and aggravates asbestosis, but there is no association between smoking and mesothelioma independent of the asbestos exposure

  • Asbestos exposure occurs from work in

    • Mining

    • Milling

    • Manufacturing

    • Shipyard work

    • Insulation

    • Brake linings

    • Building construction and demolition

    • Roofing materials

    • Other asbestos-containing products (eg, some "popcorn" ceilings)

Clinical Findings

Symptoms and Signs

  • Insidious onset of shortness of breath, nonpleuritic chest pain, and weight loss

  • Physical findings include

    • Dullness to percussion

    • Diminished breath sounds

    • Clubbing of fingers (some cases)

  • Malignant pleural mesothelioma progresses rapidly as the tumor spreads along the pleural surface to involve the pericardium, mediastinum, and contralateral pleura

  • Tumor may eventually extend beyond the thorax to involve abdominal lymph nodes and organs

Differential Diagnosis

  • Chronic organized empyema

  • Sarcoma

  • Metastatic tumor to the pleura, especially adenocarcinoma

  • Malignant fibrosing histiocytoma

  • Other causes of pleural effusion (see Pleural Effusion)

Diagnosis

Laboratory Tests

  • Pleural fluid analysis often reveals a hemorrhagic exudate

  • Cytologic tests of pleural fluid are often negative

  • Soluble mesothelin-related peptide and fibulin-3 may be found in serum or pleural fluid (or both) of patients with malignant mesothelioma

Imaging Studies

  • Radiographic findings

    • Nodular, irregular, unilateral pleural thickening

    • Varying degrees of unilateral pleural effusion

  • CT helps determine the extent of pleural involvement

  • PET-CT helps differentiate benign from malignant pleural disease, improve staging accuracy, and identify candidates for aggressive surgical approaches

Diagnostic Procedures

  • Thoracentesis

  • Video-assisted thoracoscopic surgery (VATS) biopsy is usually necessary to obtain an adequate specimen for histologic diagnosis; special stains and electron microscopy may be needed to confirm the diagnosis

Treatment

Medications

  • Chemotherapy: pemetrexed and ...

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