Benign multicystic mesothelioma: a case report of three sisters

• Published in: Rare tumors Vol. 1; no. 2; pp. e46 - 145
• Main Authors: Bernstein, Eve M, Tate, Alison, Silasi, Dan Arin, Rutherford, Thomas
• Format: Journal Article
• Language: English
• Published: England PAGEPress Publications 28.12.2009; SAGE Publishing
• Subjects: Benign multicystic mesothelioma; Case Report; Genetic or familial association; Gynecology; Multilocular inclusion cyst; Oncology; Peritoneal inclusion cyst; Peritoneal mesothelioma; Tumors; gynecology; tumors; benign multicystic mesothelioma; multilocular inclusion cyst; peritoneal inclusion cyst; peritoneal mesothelioma; oncology; genetic or familial association
• ISSN: 2036-3613; 2036-3605; 2036-3613
• DOI: 10.4081/rt.2009.e46

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.2 The mesothelial origin was later confirmed by electron micro-scopy by Mennemeyer and Smith in 1979.3 To date, there are approximately 140 cases of BMCM reported in the literature.4 This disease primarily occurs in pre-menopausal women and is associated with a history of pelvic inflammatory disease, prior abdominal surgery, and endometriosis.4,5 The pathogenesis of this disease remains controversial, with possible etiologies including a neoplastic versus a reactive process.5 In the literature, a few case reports discuss a possible genetic or familial association with BMCM.6 Specifically, one report describes a man with familial Mediterranean fever who developed BMCM. Although familial Mediter-ranean fever is associated with malignant mesothelioma, he had only BMCM, and did not suffer from malignant mesothelioma.6 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.