An intra-abdominal small round cell neoplasm with features of primitive neuroectodermal and desmoplastic round cell tumor and a EWS/FLI-1 fusion transcript.
Hum Pathol
; 28(4): 502-9, 1997 Apr.
Article
em En
| MEDLINE
| ID: mdl-9104953
ABSTRACT
We report an intra-abdominal round cell tumor in a young man which exhibited the light and electron microscopic appearance of a peripheral primitive neuroectodermal tumor (PNET), in addition to the clinical and topographic characteristics, desmoplasia and a complex immunophenotypic profile of the intra-abdominal desmoplastic round cell tumor (DSRCT). Reverse transcription polymerase chain reaction revealed a EWS/FLI-1 fusion transcript as in PNET/Ewing's sarcoma, instead of the EWS/WT1 transcript of DSRCT. The tumor was also strongly positive for the mic2 protein. This is a unique case of a hybrid tumor arising in the peritoneal cavity of a young male. The existence of such a hybrid tumor in this location suggests that DSRCT and PNET may be related and possibly share a common histogenesis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sarcoma de Ewing
/
Proteínas de Fusão Oncogênica
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Proteínas Proto-Oncogênicas
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Tumores Neuroectodérmicos Primitivos
/
Carcinoma de Células Pequenas
/
Neoplasias Abdominais
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Ano de publicação:
1997
Tipo de documento:
Article