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WDHA综合征患者中同时表达血管活性肠肽(VIP)和血管活性肠肽1型受体(VPAC1)的血管活性肠肽瘤

VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome.

作者信息

Nakayama Shoko, Yokote Taiji, Kobayashi Kichinosuke, Hirata Yuji, Hiraiwa Tetsuya, Komoto Izumi, Miyakoshi Kazuho, Yamakawa Yoshiko, Takubo Takayuki, Tsuji Motomu, Imamura Masayuki, Hanafusa Toshiaki

机构信息

Department of Internal Medicine (I), Osaka Medical College, 2-7 Daigakumachi, Takatsuki City, Osaka 569-0801, Japan.

出版信息

Endocrine. 2009 Apr;35(2):143-6. doi: 10.1007/s12020-009-9146-6. Epub 2009 Jan 30.

DOI:10.1007/s12020-009-9146-6
PMID:19184565
Abstract

We report a case of VIPoma in a 72-year-old female patient who presented with excessive diarrhea, severe hypokalemia, and acidemia. She had been referred to our hospital three times because of severe diarrhea. No primary tumor site was found by conventional techniques, including contrast-enhanced CT and MRI, angiography, endoscopy, and positron emission tomography (PET), but a tumor was subsequently found in the head of the pancreas by octreotide scanning. Her diarrhea diminished dramatically after octreotide treatment, while her diarrhea has ceased without the therapy of octreotide at the first admission in the course of 2 years of her disease. Immunohistochemial analysis of the excised tumor tissue revealed the expression of both vasoactive intestinal peptide (VIP) and VIP and pituitary adenylate cyclase-activating peptide 1 (VPAC1) receptors. This is the first case report of a VIPoma that immunostains for VIP and VPAC1 receptors and indicates that abundant VIP produced by VIPoma might inhibit its growth and reduce VIP secretion via the VPAC1 receptor in vivo.

摘要

我们报告一例72岁女性患者的血管活性肠肽瘤(VIPoma),该患者表现为腹泻过度、严重低钾血症和酸血症。她因严重腹泻曾三次转诊至我院。通过包括增强CT、MRI、血管造影、内镜检查和正电子发射断层扫描(PET)在内的传统技术未发现原发肿瘤部位,但随后通过奥曲肽扫描在胰头发现了肿瘤。奥曲肽治疗后她的腹泻显著减轻,在疾病的2年病程中,首次入院时未经奥曲肽治疗腹泻已停止。对切除的肿瘤组织进行免疫组织化学分析显示血管活性肠肽(VIP)和VIP及垂体腺苷酸环化酶激活肽1(VPAC1)受体均有表达。这是首例对VIP和VPAC1受体进行免疫染色的VIPoma病例报告,表明VIPoma产生的大量VIP可能在体内通过VPAC1受体抑制其生长并减少VIP分泌。

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