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家族性大腸腺腫症では,上部消化管病変が高頻度に合併することが最近の研究で明らかになってきた,欧米ではGardner症候群に随伴した十二指腸乳頭部癌の例が幾つか報告されている1)~8).本邦では胃,十二指腸,小腸などの腺腫の随伴する例が報告されているが9)~12),大腸以外の癌合併例の報告は極めて少ない.最近われわれは家族性大腸腺腫症で結腸全摘出術施行後7年目に早期十二指腸乳頭部癌および十二指腸腺腫を認めた本邦第1例目と思われる症例を経験したので若干の文献的考察を加えて報告する.
Recently we have treated a patient with early cancer of the ampulla of Vater and duodenal adenoma which developed seven years after total colectomy for familial polyposis coli. This case may be the first one reported in Japan, while 11 cases of such case have been reported in the previous literature in Europe and America. The patient was a 37-year-old man. His family history shows that his couson aged 54 had colonic adenomas accompanied with cancer of the stomach and that his nephew aged 26 was also operated on for colonic adenomas at this department. This patient underwent total colectomy with ileoproctostomy in June 1971. In January 1978 he had upper abdominal pain.
In August he experienced the sense of fullness in the upper abdominal region and back pain. In November elevated urine amylase level was noted. Anemia and jaundice were not observed.
Roentgenographic examination of the stomach and duodenum revealed the filling defects of the tip of the thumb in size at the region where was considered as the papilla of Vater. Endoscopic examination showed a tumor mass with a shallow depression at the same region.
Biopsy of that region showed tubular adenocarcinoma. On 14th February 1979, the pancreatoduodenectomy was performed. Cancer lesion measuring 1.8×1.5 cm was identified histologically as tubular adenocarcinoma of the ampulla of Vater. A duodenal adenoma was found at the separate position from the cancer but adjacent to it. The patient progressed satisfactorily after the operation. At present, two years later, he shows no sign of recurrence and is working. With this case, we keenly felt the importance of fol-low-up observations, particularly periodic examinations on the upper digestive tract after the operation for familial polyposis coli.
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