一例导致WDHA综合征的分泌胰多肽的胰腺肿瘤
A Pancreatic Polypeptide-Producing Pancreatic Tumor Causing WDHA Syndrome.
作者信息
Amrilleva Vera, Slater Emily P, Waldmann Jens, Bonorden Dorothee, Fendrich Volker
机构信息
Department of Surgery, Philipps University Marburg, Marburg, Germany.
出版信息
Case Rep Gastroenterol. 2008 Jul 9;2(2):238-43. doi: 10.1159/000142739.
We report the case of a 46-year-old female patient with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome caused by a pancreatic polypeptide-producing tumor in the head of the pancreas. Whereas VIP and other pancreatic endocrine hormones were in the normal range, only serum levels of pancreatic polypeptide were elevated. Imaging studies identified a pancreatic tumor in the head of the gland. After laparotomy, the tumor of 3 cm in size was enucleated. Final pathology documented a pancreatic endocrine tumor with immunohistochemical staining demonstrating the presence of pancreatic polypeptide. The patient remained cured after a follow-up of more than three years. The present case illustrates that, although rare, WDHA syndrome may be associated with a pancreatic polypeptide-secreting endocrine tumor of the pancreas.
我们报告了一例46岁女性患者，其患有由胰腺头部产生胰多肽的肿瘤引起的WDHA（水样腹泻/低钾血症/无胃酸）综合征。虽然血管活性肠肽和其他胰腺内分泌激素在正常范围内，但仅血清胰多肽水平升高。影像学检查发现胰腺头部有一个肿瘤。剖腹手术后，摘除了一个3厘米大小的肿瘤。最终病理证实为胰腺内分泌肿瘤，免疫组化染色显示存在胰多肽。经过三年多的随访，患者保持治愈状态。本病例表明，尽管罕见，但WDHA综合征可能与胰腺分泌胰多肽的内分泌肿瘤有关。
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