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Carcinoma of Ampulla of Vater and Recurrent Desmoid Tumors in Gardner's Syndrome: A Case Report

Gardner's症候群合併壺腹部癌及反覆性腹部纖維瘤:一病例報告

摘要


Gardner's症候群乃具有大腸息肉、顱骨及下顎骨之骨瘤、軟組織腫瘤等特徵,也會引起一些大腸外之臨床症狀,特別是壺腹部周圍之腫瘤及腹部纖維瘤。我們報告一例36歲女性患者,有Gardner's症候群及反覆性腹部纖維瘤之病史,以兩天發燒及全身倦怠表現。因嚴重貧血,故接受胃鏡檢查,於胃鏡檢查時意外發現在十二指腸乳頭處有一息肉樣之腫瘤合併出血,經由內視鏡切片檢查發現為一腺瘤有局部惡性變化。之後黃疸發生,接受疸汁引流術。但病人不願接受壺腹部腫瘤切除手術。在住院第25天出院。然而出院後未回診,據說一年後在其他醫院因病去世。

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並列摘要


Gardner's syndrome is characterized by colonic polyps, osteomas of the skull and the mandible, and soft tissue tumors. Patients with this syndrome can also develop extracolonic manifestations such as periampullary tumors and desmoid tumor of the abdomen. We report a 36 year-old female with Gardner's syndrome and recurrent desmoid tumors of the abdomen, presenting with fever and malaise for 2 days. Upper gastrointestinal endoscopy for marked anemia showed a bleeding polypoid tumor in the ampulla of Vater. Histopathology yielded a tubular adenoma with focal carcinomatous change. Obstructive jaundice developed later and biliary drainage was carried out. Surgery for the ampullary tumor was advised but the patient refused. The patient improved and was discharged after 25 days of hospitalization but she was reported to die one year later.

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