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PERITONEAL CYSTIC MESOTHELIOMA
Synonym(s):
-Multilocular peritoneal inclusion cyst
-Multicystic mesothelioma
-Benign multicystic peritoneal mesothelioma
Disease definition :
Peritoneal cystic mesothelioma is a rare benign tumor characterized by the formation of intra-abdominal multilocular cystic masses.
Epidemiology:
Less than 150 cases have been reported so far. It occurs more frequently in women of reproductive period.
Clinical description:
Symptoms include lower abdominal or pelvic pain, abdominal swelling, constipation, presence of a palpable mass, absence of menstruation, urinary hesitancy, dyspareunia, and more rarely weight loss. In women, the masses are generally located in the peritoneal surface of the uterus and rectum, while in men they are generally located in the peritoneal surface of the bladder and rectum. The tumor can also spread into the upper portions of the peritoneal cavity.
Etiology:
Peritoneal cystic mesothelioma seems to originate from the peritoneal mesothelium. However, etiology is unknown. There is no relation with asbestos exposure. It occurs frequently in women who have history of previous surgery, pelvic inflammatory or endometriosis. Chronic peritoneal irritation may be a precipating stimulus.
Diagnostic methods:
Diagnosis is based on abdominal and pelvic ultrasonography, computerized tomography and laparoscopic examination that reveal abdominal septated grapelike multicystic structures. Biopsy shows vascularized, translucent, fluid-filled thin-walled cysts composed of loose connective tissue. Immunohistochemistry helps to confirm the diagnosis by showing the mesothelial origin of the cell lining.
Differential diagnosis:
Differential diagnosis includes pseudomyxoma peritonei, lymphangioma (see these terms), complex loculated ascites, and other cystic neoplastic lesions.
Management and treatment :
Treatment is surgical and is based on cysts resections. In case of recurrence or in front-line, the combination of cytoreductive surgery with hyperrthermic intraperitoneal chemotherapy (HIPEC) on selected patients (young, good general status) should be considered and improve disease-free survival.
Prognosis :
With surgery alone, disease invariably recurs or persists. Evolution to invasive or malignant disease has been described.
Prognosis is usually good. All patients treated with combined treatment are alive at 5 years.
Professor Olivier Glehen (October 27, 2010)