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Introduction

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Most malignant diseases of the pleura present with a unilateral pleural effusion or thickening. Malignant diseases include the very common secondary malignancy metastatic to the pleura, the less common lung cancer patient with pleural carcinomatosis, and the uncommon patient with primary malignant pleural mesothelioma (MPM). The low morbidity and accuracy of diagnostic thoracoscopy and pleural biopsy have dramatically increased our understanding and treatment of these diseases. This section of chapters provides important details and management of this group of primary malignant and secondary malignant diseases of the pleura.

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Ironically, much of our insight has been gained by trying to understand the least common yet deadliest of this group of diseases: pleural mesothelioma. The current incidence of pleural mesothelioma within the United States is 2000 to 3000 cases per year as compared with esophageal and lung cancer, which are at least four and 50 times more common, respectively.1 Few physicians will treat more than a handful of cases of MPM over the course of their professional careers. Even fewer academic centers in North America and Europe have been able to acquire a collective experience large enough to develop new treatment protocols for this devastating disease. The Brigham and Women's Hospital (BWH) and Dana-Farber Cancer Institute (DFCI) in Boston, Massachusetts, have gained a large experience treating MPM over the last 25 years.

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The recognition of mesothelioma as a cancer and the development of treatment options are recent developments in the context of medical history. In 1950, Stout described malignant mesothelioma as a separate pathologic entity.2 Stout also described the three histologic subsets: epithelial, fibrosarcomatous, and mixed.

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In 1960, Wagner et al.3 published the first mesothelioma case series, reporting on 33 patients from a South African asbestos mining town with known occupational and environmental crocidolite exposure. In the 1970s, a landmark study by Selikoff4 was published which established a firm link between asbestos exposure and mesothelioma. The investigators followed 17,800 asbestos insulation workers in the United States and Canada for a period of up to 50 years. They found that the incidence of mesothelioma within this group increased rapidly starting 20 to 25 years after the first exposure. Peak incidence occurred at 40 to 45 years after exposure. Seven percent of all deaths in this group of asbestos workers were due to mesothelioma, a shockingly high incidence for a rare cancer. Family members of asbestos workers also have a substantial increased risk, termed “bystander risk,” thought to be secondary to exposure to hair and clothes brought into the home.5

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Early efforts at surgical and nonsurgical treatments were disappointing. Worn6 published one of the first series of patients undergoing extrapleural pneumonectomy (EPP) in 1974 reporting a 5-year survival rate of 10% and a median survival of 19 months. Butchart et al.7 published their initial experience with EPP for maximal surgical debulking of pleural mesothelioma in 1976. EPP ...

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