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.
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Authors | T E Lans, G H van Ramshorst, J J Hermans, M A den Bakker, T C K Tran, G Kazemier |
Journal | Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
(J Gastrointest Surg)
Vol. 13
Issue 2
Pg. 389-92
(Feb 2009)
ISSN: 1873-4626 [Electronic] United States |
PMID | 18213505
(Publication Type: Case Reports, Journal Article)
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Chemical References |
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Topics |
- Adult
- Anti-Bacterial Agents
(therapeutic use)
- Chylous Ascites
(diagnosis, etiology, therapy)
- Drainage
- Female
- Humans
- Perivascular Epithelioid Cell Neoplasms
(complications, pathology, surgery)
- Postoperative Complications
- Retroperitoneal Neoplasms
(complications, pathology, surgery)
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