Diagnosis and treatment of VlPoma in a female patient

Diagnosis and treatment of VlPoma in a female patient
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DOI:
10.1097/00042737-200601000-00017
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发表时间:
2006-01-01
影响因子:
2.1
通讯作者:
Schrijver, Gideon
Schrijver, Gideon
中科院分区:
医学4区
文献类型:
--
作者:
Remme, Carol Ann;de Groot, Gerrit H.;Schrijver, Gideon

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我们报告一位83岁女性患者的VIPoma病例,她表现为频繁且过度的腹泻、肌肉无力和严重的低钾血症。腹部计算机断层扫描(CT)显示胰腺体内有一个4x6厘米的肿块。实验室分析显示,血管活性肠多肽(VIP;153pmo1/l)和胰腺多肽(161pmo1/1)水平均升高。考虑到患者的年龄、身体状况和肿瘤大小,没有进行手术切除。患者接受了长效奥曲肽的治疗,之后她的症状减轻了。经过24个月的随访,患者的身体状况良好,没有任何进一步的严重胃肠道症状。VIPoma综合征是由一种神经内分泌肿瘤引起的,通常位于胰腺,分泌VIP,导致严重腹泻、脱水和低钾血症。治疗方案包括切除肿瘤、化疗或使用生长抑素类似物减轻症状。我们对这种罕见综合征的发生率、病理生理学、诊断、治疗策略和预后进行了概述。
We report a case of VIPoma in an 83-year-old female patient, who presented with frequent and excessive diarrhoea, muscle weakness, and severe hypokalaemia. Abdominal computed tomography (CT) revealed a 4 x 6 cm mass in the body of the pancreas. Laboratory analysis showed elevated levels of both vasoactive intestinal polypeptide (VIP; 153 pmol/1) and pancreatic polypeptide (161 pmol/1). In view of the patient's age, physical condition, and tumour size, surgical resection was not performed. The patient was treated with a long-acting octreotide, after which her symptoms diminished. After 24 months of follow-up, the patient remained in good physical condition without any further serious gastrointestinal symptoms. The VIPoma syndrome is caused by a neuroendocrine tumour, usually located in the pancreas, which secretes VIP, causing severe diarrhoea, dehydration and hypokalaemia. Treatment options include resection of the tumour, chemotherapy or the reduction of symptoms with somatostatin analogues. We provide an overview of the incidence, pathophysiology, diagnosis, treatment strategies, and prognosis of this rare syndrome.