Cronkhite-Canada Syndrome Associated with Gastric Carcinoid Tumor: Report of a Case

Cronkhite-Canada症候群合併有類癌的個案是很少见的。在此，我们报導一個七十六歲的男性，因爲反覆胃痛及胃脹而就醫。病人的異常理學检查包括秃頭、指甲龜裂及普遍的全身色素沉著。上消化内视镜检查及小腸攝影，發现有多發性的胃及小腸息肉；同時胃镜检查也發現於胃寶有一個1.8公分之黏膜下腫瘤。胃息肉的組織學检查顯示属發炎性息肉，而胃内之黏膜下腫瘤则被诊断爲類癌，其病理學之特徵爲上皮性腫瘤合讲有典型的神经内分泌樣的分化及G細胞的增生。大腸镜也看到多發性的息肉，大肠息肉的切片有典型Cronkhite-Canada症候群發炎性大腸息肉的組織學變化。全腹部的電腦斷層检查，看到的幽門前區的胃壁有增厚但並無淋巴结腫大。除了血清中的gastrin-17值偏高以外，其他的神经内分泌腫瘤相關的特殊血液及尿液检查结果皆正常。雖然我们建議病人開刀，但病人拒絕且失去追蹝有雨年之久。最後，此病患因持續有嚴重的蛋白質流失性腸炎造成之营養不良、嚴重感染、敗血症而引發多重器官衰竭而死亡。

關鍵字

秃頭； Cronkhite-Canada症候群；胃類癌；蛋白質流失性腸病變

並列摘要

Cronkhite-Canada syndrome associated with gastric carcinoid tumor is very rare. We report a 76-year-old male presented with the chief complaints of epigastric pain and abdominal distention. His abnormal physical findings included alopecia, splitting of nail bed and generalized hyperpigmentation. Both upper gastrointestinal endoscopy and small intestinal series revealed multiple polypoid lesions in the stomach and the small intestine. In addition, a submucosal mass was also noted over the antrum. The histopathologic diagnosis of his gastric polyps was inflammatory polyps. Nervertheless, his gastric submucosal lesion was proved to be a carcinoid tumor. In addition, several sessile polyps were also found by colonoscopy. Although high serum level of gastrin-17, other special endocrine studies including levels of 24-hours urinary 5-HIAA excretion, plasma metanephrines, catecholamines, vanillylmandelic acid were all within normal limits. Surgical intervention was suggested, but he refused and was lost to follow-up for two years. Finally, he died from aspiration pneumonia with multiple organ failure due to protein-losing enteropathy and severe malnutrition.

並列關鍵字

alopecia ； Cronkhite-Canada syndrome ； gastric carcinoid ； protein-losing enteropathy

國際替代計量

Cronkhite-Canada Syndrome Associated with Gastric Carcinoid Tumor: Report of a Case

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