Malignant pleural mesothelioma

Malignant peritoneal mesothelioma

Malignant pleural mesothelioma

Mesothelioma Treatment guidelines

Malignant mesothelioma arises from the serosal lining of the pleural, peritoneal and pericardial cavities. Diffuse malignant peritoneal mesothelioma (MPM) accounts for 25% of cases, with a very low incidence in France (approximating 0.3/100,000). The role of asbestosis in its onset is unclear.

The most frequent clinical presentation is abdominal pain and distention caused by accumulation of tumors and ascitic fluid. Exploratory coelioscopy is the most useful exploration method to obtain the diagnosis based on biopsy specimens and to provide a precise description of the extent of the disease. Macroscopically, MPM is characterized by thousands of whitish tumor nodules, variable in size and consistency that may coalesce
to form plaques, masses or are layered out uniformly covering the entire peritoneal surface. Histologically, the diagnosis is frequently difficult, and only specific immunostains (calretinin and WT-1 are positive mesothelial markers, and polyclonal CEA and Ber-EP4 are negative markers) allow a definitive diagnosis of MPM. Moreover, one is frequently faced with a broad spectrum of architectural patterns from one microscopic field to another, making it difficult to classify each case as a distinct histological type. Conventional Mesothelioma treatment is a combination of systemic chemotherapy (similar to that administered for pleural mesothelioma) and palliative surgery, yielding a median survival uniformly
close to one year. However, it is well known that most MPM remain localized within the abdomino-pelvic cavity throughout their natural history, as evidenced in autopsy series. It is
therefore logical to consider that an aggressive local Mesothelioma treatment could be more efficient. During the last 5 years, a cytoreductive surgical approach combined with HIPEC has been used by a few teams and has resulted in dramatically improved median survival
approaching 5 years for patients undergoing such Mesothelioma treatment.

Malignant pleural mesothelioma 

Mesothelioma of the pleura was first described in a case of asbestosis in 1943. This was
considered to be an exceptionally rare disease of uncertain etiology until 1960 when
Christopher Wagner working at the Pneumoconiosis Bureau in South Africa carefully
documented a large series ofpatients with this disease. Among his first 33 cases ofmalignant mesothelioma 32 had been exposed to crocidolite asbestos (73). Major asbestos mining in South Africa began in 1930 and the incidence of malignant mesothelioma increased dramatically by 1960. A similar increase is expected in the U.S.A., as major asbestos usage began in 1950 (52). In 1982 a report from the Connecticut Tumor Registry suggested a rising incidence of mesothelioma (41). It is no longer open to doubt that asbestos exposure plays a highly significant role in the etiology of malignant pleural mesothelioma nor that the dose of asbestos is less than that which causes asbestosis. The time from exposure to the development of malignant pleural mesothelioma varies from a few to many years with most patients developing tumor IO-20 years after exposure.
Mesotheliomas are tumors which arise from the mesothelium covering the body cavities.
It arises most frequently in the pleura or peritoneum, but also in the pericardium and
tunica vaginalis testis. The current overview is confined to the common malignant mesothelioma arising in the pleural cavity.

The natural history of malignant pleural mesothelioma was first clearly delineated by
Wagner when studying asbestosis. He clearly described the findings in 78 patients;
their exposure, radiological picture and survival from initial symptoms (median more
than 9 months) in his doctoral thesis more than a quarter of a century ago. In 1984 Law
et al. reported the natural history of a series of 64 patients treated with supportive
care only. The median survival (from the onset of symptoms) was 18 months. Survival
exceeded 4 years in seven patients and 5 years in four patients; one patient survived 16
years. This study confirmed previous reports of occasional prolonged survival of untreated
patients. It is evident in any series of patients that occasional long-term survivors may be found. In treated patients this may be falsely attributed to the effects of  Mesothelioma treatment.

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