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表现为胰岛素瘤、胃泌素瘤和球后十二指肠溃疡的1型多发性内分泌腺瘤病:病例报告

Multiple endocrine neoplasia type 1 presenting as insulinoma, gastrinoma, and postbulbar duodenal ulcers: report of a case.

作者信息

Murakami S, Satomi A, Ishida K, Matsuki M, Koda H, Miura S

机构信息

Second Department of Surgery, Saitama Medical School, Japan.

出版信息

Surg Today. 1995;25(9):848-51. doi: 10.1007/BF00311466.

Abstract

We report herein the unusual case of a 55-year-old man with multiple endocrine neoplasia type 1 presenting as hyperparathyroidism, hyperpituitarism, insulinoma, and gastrinoma with postbulbar duodenal ulcers. The patient was referred to our hospital for further investigations of nephrolithiasis, acromegaly, and hematemesis. Laboratory studies showed high serum levels of calcium, parathyroid hormone, growth hormone, insulin, gastrin, and prolactin. Computed tomography of the cranial cavity demonstrated an enlargement of the serra turcica and swelling of two parathyroid glands in the neck. Computed tomography and angiography of the abdomen also showed a mass in the head of the pancreas. Endoscopy demonstrated reflux esophagitis, erosive gastritis, and multiple postbulbar duodenal ulcers. We diagnosed this patient as having multiple endocrine neoplasia type 1, with concomitant lesions of the pituitary gland, parathyroid glands, and islet cells of the pancreas. Following excision of the two enlarged parathyroid glands, his serum calcium and parathyroid hormone levels fell to within the normal range. Thereafter, a total gastrectomy was performed to alleviate the frequent bleeding from the upper gastrointestinal tract. However, resection of the pancreatic mass could not be performed owing to severe inflammation around the duodenum, probably induced by the postbulbar duodenal ulcers.

摘要

我们在此报告一例罕见病例,一名55岁男性患有1型多发性内分泌腺瘤病,表现为甲状旁腺功能亢进、垂体功能亢进、胰岛素瘤和胃泌素瘤,并伴有球后十二指肠溃疡。该患者因肾结石、肢端肥大症和呕血被转诊至我院进一步检查。实验室检查显示血清钙、甲状旁腺激素、生长激素、胰岛素、胃泌素和催乳素水平升高。头颅计算机断层扫描显示蝶鞍增大,颈部两个甲状旁腺肿大。腹部计算机断层扫描和血管造影也显示胰头有一个肿块。内镜检查显示反流性食管炎、糜烂性胃炎和多个球后十二指肠溃疡。我们诊断该患者患有1型多发性内分泌腺瘤病,同时伴有垂体、甲状旁腺和胰腺胰岛细胞病变。切除两个肿大的甲状旁腺后,他的血清钙和甲状旁腺激素水平降至正常范围。此后,进行了全胃切除术以缓解上消化道频繁出血。然而,由于十二指肠周围严重炎症(可能由球后十二指肠溃疡引起),无法切除胰腺肿块。

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