Carney Triad in an Adult with Aggressive Behavior: The First Case in Korea
Yonsei Medical Journal
;
: 709-712, 2009.
Artigo
em Inglês
| WPRIM
| ID: wpr-222142
ABSTRACT
Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Paraganglioma
/
Neoplasias Retroperitoneais
/
Neoplasias Gástricas
/
Tumores do Estroma Gastrointestinal
/
Complexo de Carney
/
Coreia (Geográfico)
Limite:
Adulto
/
Feminino
/
Humanos
País/Região como assunto:
Ásia
Idioma:
Inglês
Revista:
Yonsei Medical Journal
Ano de publicação:
2009
Tipo de documento:
Artigo
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