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Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma
- Source :
- Journal of Gastrointestinal Surgery, 13(2), 389-392. Springer New York
- Publisher :
- Springer Nature
-
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.
- Subjects :
- Adult
Abdominal pain
Pathology
medicine.medical_specialty
Chyle
Abdominal Fluid
business.industry
Perivascular Epithelioid Cell Neoplasms
Gastroenterology
Perivascular Epithelioid Cell
Anti-Bacterial Agents
Parenteral nutrition
Postoperative Complications
Chylous ascites
Medicine
Drainage
Humans
Female
Surgery
Retroperitoneal Neoplasms
medicine.symptom
business
Complication
Epithelioid cell
Chylous Ascites
Subjects
Details
- Language :
- English
- ISSN :
- 1091255X
- Volume :
- 13
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Journal of Gastrointestinal Surgery
- Accession number :
- edsair.doi.dedup.....3c53af5eba6c930f5cc55da473fad9cf
- Full Text :
- https://doi.org/10.1007/s11605-007-0462-y