Perivascular epithelioid cell tumor of the retroperitoneum in a young woman resulting in an abdominal chyloma.
J Gastrointest Surg
; 13(2): 389-92, 2009 Feb.
Article
en En
| MEDLINE
| ID: mdl-18213505
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Complicaciones Posoperatorias
/
Neoplasias Retroperitoneales
/
Ascitis Quilosa
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Neoplasias de Células Epitelioides Perivasculares
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Gastrointest Surg
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2009
Tipo del documento:
Article
País de afiliación:
Países Bajos
Pais de publicación:
Estados Unidos