A Case of Sporadic Gardner's Syndrome Accompanied with a Giant Mesenteric Desmoid Tumor with APC Gene Mutation

DOI Web Site 2 Citations 57 References

Bibliographic Information

Other Title
  • 経過中に腸間膜巨大デスモイド腫瘍が発生しＡＰＣ遺伝子異常が確認された孤発性Ｇａｒｄｎｅｒ症候群の１症例

Search this article

Description

A 38-year-old Japanese male was admitted to our hospital for abdominal expansion and a giant tumor. At 34 years old, he was diagnosed with FAP (familial adenomatous polyposis) because more than 100 polypoid lesions from 3 to 10mm in diameter were identified in the upper and lower GI tract by endoscopy. One of the biopsied specimens from the colonic polypoid lesions showed adenocarcinoma, and he was followed up once a year by endoscopy. On admission, a CT scan showed a giant tumor 28cm in diameter occupying the entire abdominal cavity. APC gene mutation was detected. The tumor and the involved tissue were resected and histopathologically diagnosed as a mesenteric desmoid tumor. Periodical examination by endoscopy and CT scan after the operation has shown no recurrence for more than five years. Gardner's syndrome has been reported to accompanied with a desmoid tumor in 3.5-17.5% of cases; however, the clinical features of sporadic Gardner's syndrome remains unclear, and the relationship between the APC gene and the disease requires further investigation.<br>

Journal

Citations (2)*help

See more

References(57)*help

See more

Keywords

Details 詳細情報について

Report a problem

Back to top