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.

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Keywords Chylous, PEComa, Retroperitoneum
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Journal Journal of Gastrointestinal Surgery
Lans, T, van Ramshorst, G.H, Hermans, J.J, den Bakker, M.A, Tran, T.C, & Kazemier, G. (2009). Perivascular epithelioid cell tumor of the retroperitoneum in a young woman resulting in an abdominal chyloma. Journal of Gastrointestinal Surgery, 13(2), 389–392. doi:10.1007/s11605-007-0462-y