title: Perivascular epithelioid cell tumor of the retroperitoneum in a young woman resulting in an abdominal chyloma.
abstract: Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm which appears to have predominancy for young, frequently Asian, women. The neoplasm is composed chiefly of HMB-45-positive epithelioid cells with clear to granular cytoplasm and usually showing a perivascular distribution. These tumors have been reported in various organs under a variety of designations. Malignant PEComas exist but are very rare. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. We report a retroperitoneal PEComa discovered during emergency surgery for abdominal pain in a 28-year-old Asian woman. The postoperative period was complicated by chylous ascites that was initially controlled by a wait-and-see policy with total parenteral nutrition. However, the chyle production gradually increased to more than 4 l per day. The development of a bacterial peritonitis resulted in cessation of production of abdominal fluid permitting normal nutrition without chylous leakage. Effective treatment for this rare complication of PEComa is not yet known; therefore, we have chosen to engage in long-term clinical follow-up.
authors.affiliation: Department of Surgery, Erasmus MC-University Medical Center, 'sGravendijkwal 230, 3015 GD Rotterdam, The Netherlands. T.Lans@ErasmusMC.NL
authors.forename: T E
authors.initials: TE
authors.lastname: Lans
authors.forename: G H
authors.initials: GH
authors.lastname: van Ramshorst
authors.forename: J J
authors.initials: JJ
authors.lastname: Hermans
authors.forename: M A
authors.initials: MA
authors.lastname: den Bakker
authors.forename: T C K
authors.initials: TC
authors.lastname: Tran
authors.forename: G
authors.initials: G
authors.lastname: Kazemier
mesh_terms: D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002915:Chylous Ascites / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D004322:Drainage; D005260:Female; D006801:Humans; D054973:Perivascular Epithelioid Cell Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications; D012186:Retroperitoneal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery*
chemical_list: D000900:Anti-Bacterial Agents