Mesotheliomas are almost universally considered a fatal neoplasm, and until recently the treatment options were very limited and ineffective. The overall median survival was only 1 year. Nevertheless, a few mesothelioma cases of long-term survivors have been reported (longest survival reported is 19 years.
After the introduction of cytoreductive surgery followed by intraperitoneal hyperthermic perfusion, a significant prolongation in the median survival has been achieved, with approximately half the patients alive at 5 years. Tumor histopathology, previous surgical mesothelioma score, lesion size, gender, distribution (assessed with Gilly classification and peritoneal cancer index), and completeness of cytoreduction score represent the main prognostic index.
Multicystic mesothelioma and well-differentiated peritoneal mesothelioma typically occur in the peritoneum of women without a history of asbestos exposure and show low malignant potential. Multicystic mesothelioma predominantly affects the pelvic peritoneum of young women, has a high tendency to recur locally, but no tendency to metastasis, and consequently requires only surgical treatment. Some theories link this type of mesothelioma to fibromatoses, suggesting a reactive hyperplastic process more than a neoplastic one A familial form of multicystic mesothelioma has also been described. Sarcomatoid mesothelioma, although rare, is the most aggressive histotype, with a median survival reported of only 3.8 months.
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