Benign multicystic mesothelioma: a case report of three sisters

Benign multicystic mesothelioma (BMCM) is a rare tumor of the abdomen-peritoneum of unknown etiology. This benign tumor was initially described by Plaut in 1928 when he observed loose cysts in the pelvis during a surgery for a uterine leiomyoma. The mesothelial origin was later confirmed by electron microscopy by Mennemeyer and Smith in 1979. To date, there are approximately 140 cases of BMCM reported in the literature. This disease primarily occurs in pre-menopausal women and is associated with a history of pelvic inflammatory disease, prior abdominal surgery, and endometriosis. The pathogenesis of this disease remains controversial, with possible etiologies including a neoplastic versus a reactive process. In the literature, a few case reports discuss a possible genetic or familial association with BMCM. Specifically, one report describes a man with familial Mediterranean fever who developed BMCM. Although familial Mediterranean fever is associated with malignant mesothelioma, he had only BMCM, and did not suffer from malignant mesothelioma. A genetic evaluation and chromosomal analysis were not able to identify a specific genetic cause of the family’s pattern of disease. This case report describes two female siblings diagnosed with BMCM. In addition, a third sister also had findings consistent with BMCM, however, the discrete histological diagnosis was never confirmed.