Ono C, Iwama T, Mishima Y
Second Department of Surgery, Tokyo Medical and Dental University, Japan.
Jpn J Surg. 1991 Mar;21(2):234-40. doi: 10.1007/BF02470915.
A 63 year old female with familial adenomatous polyposis (FAP) complicated by carcinoma of the thyroid and ampulla of Vater and a nonfunctioning adrenocortical adenoma is reported herein. We reviewed 23 cases of duodenal or periampullary carcinoma, 10 cases of thyroid carcinoma and 4 cases of adrenal neoplasm presented in the Japanese literature. From studying these cases, we identified that FAP patients had a high risk of developing periampullary carcinoma and consequently, it is very important to periodically examine the ampulla of Vater. Thyroid carcinomas in patients with FAP were well differentiated carcinomas and occurred earlier than in the general population. The use of medical imaging equipment with mean that insidious abdominal tumors in cases of FAP will be detected more frequently in the future and as patients with FAP have a high risk of developing malignancies, surgery should be indicated for incidentally found adrenal tumors.
本文报道了1例63岁女性,患有家族性腺瘤性息肉病(FAP),并发甲状腺癌、壶腹癌和无功能肾上腺皮质腺瘤。我们回顾了日本文献中报道的23例十二指肠或壶腹周围癌、10例甲状腺癌和4例肾上腺肿瘤病例。通过对这些病例的研究,我们发现FAP患者发生壶腹周围癌的风险很高,因此,定期检查十二指肠乳头非常重要。FAP患者的甲状腺癌为高分化癌,且比普通人群发病更早。医学成像设备的使用意味着未来FAP患者隐匿性腹部肿瘤将被更频繁地检测到,而且由于FAP患者发生恶性肿瘤的风险很高,对于偶然发现的肾上腺肿瘤应考虑手术治疗。