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Localized Fibrous Tumors of Pleura

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  • • Previously called "localized mesotheliomas"

    • Arise from subpleural fibroblasts

    • Cause pulmonary nodules to pleural masses

    • Involvement of visceral pleura more common than parietal

    • Benign (70%) patterns

    • -Fibrous

      -Cellular

      -Mixed

    • Malignant (30%) patterns

    • -Tubulopapillary

      -Fibrous

      -Dimorphic

    • Behave as sarcomas

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Diffuse Malignant Pleural Mesothelioma

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  • • Most common primary tumor of pleura

    • 4 histologic variants

    • -Epithelial or rubopapillary (35-40%): Associated with pleural effusions, better prognosis

      -Fibrosarcomatous/mesenchymal (20%) "dry" mesotheliomas

      -Mixed (35-40%)

      -Undifferentiated (5-10%)

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Epidemiology

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Diffuse Malignant Pleural Mesothelioma

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  • • Strong link to asbestos exposure: 300 × increased risk

    • Amphibole fibers (crocidolite, amosite, etc) and soil silicate zeolite lodge in terminal airways migrate to pleura

    • Latency after asbestos exposure: 15-50 years

    • Right hemithorax (60%) affected more than left (35%), bilateral (5%)

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Symptoms and Signs

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Localized Fibrous Tumors of Pleura

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  • • Most asymptomatic

    • Large tumors may produce symptoms of bronchial compression

    • -Dyspnea

      -Cough

      -Chest heaviness

    • Rarely, hypoglycemia from production of insulin-like peptide (4%)

    • Clubbing, hypertrophic pulmonary osteoarthropathy (20-35%)

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Diffuse Malignant Pleural Mesothelioma

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  • • Dyspnea on exertion common

    • Chest wall discomfort common

    • Cough, fever, malaise, weight loss, dysphagia

    • Advanced disease:

    • -Pain

      -Abdominal distention

      -Pericardial tamponade

      -Superior vena cava (SVC) syndrome

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Imaging Findings

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Localized Fibrous Tumors of Pleura

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  • Chest film: Well circumscribed mass, may move with changes in position

    • Pleural effusion in 15%

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Diffuse Malignant Pleural Mesothelioma

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  • Chest film: Pleural thickening, effusion (75%), narrowing of intercostals spaces

    CT scan: Diffuse irregular pleural thickening

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Localized Fibrous Tumors of Pleura

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  • • Fine-needle aspiration (FNA) may be suggestive

    • Surgical excision often necessary for diagnosis

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Diffuse Malignant Pleural Mesothelioma

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  • • FNA usually inadequate

    • Biopsy via small incision or video-assisted thoracoscopic surgery (VATS)

    • Immunohistochemistry stains for carcinoembryonic antigen (CEA), LeuM1, B72.3, BerEP4, negative; vimentin and keratin stains positive

    • Calretinin stain (specific for mesothelial cells) usually positive

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  • • Chest x-ray

    • Chest CT scan

    • Fluorodeoxyglucose positron emission tomography(FDG-PET) scan sometimes useful

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Localized Fibrous Tumors of Pleura

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  • • Complete resection; lobectomy usually not required, wedge resection recommended if visceral pleural involved

    • If arises from parietal pleura, chest wall resection necessary

    • After excision, no further therapy needed

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Diffuse Malignant Pleural Mesothelioma

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  • • Radiation and chemotherapy alone have no impact on survival

    • Surgery: 2 approaches

    • 1. Radical pleuropneumonectomy

      2. Parietal pleurectomy with decortication: Better outcome and lower morbidity when combined with radiation therapy

    • Chemotherapy, photodynamic therapy, immunotherapy, gene therapy, intraoperative chemoradiation therapy being done at some centers under clinical trials

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