内分泌
词汇介绍
拓展阅读
解析
Vipoma
释 义 [内科] 舒血管肠肽瘤
例 句 The presence of pancreatic VIPoma should be clinically suspected in all patients with watery diarrhea, particularly when accompanied by a loss of potassium and bicarbonate and a pancreatic mass on imaging. 临床上应怀疑所有水样腹泻患者均存在胰腺舒血管肠肽瘤，特别是伴有钾和碳酸氢盐的流失和影像学检查中的胰腺肿块时。
概述
概述
舒血管肠肽瘤是一种罕见的内分泌肿瘤，即过量产生血管活性肠肽。舒血管肠肽瘤通常（约90%）发生于非β胰岛细胞的胰腺，它们有时与1型多发性内分泌肿瘤相关，大约50%-75%的舒血管肠肽瘤是恶性的，但即使它们是良性的，它们也倾向于引起特定的综合征：如引起了严重而慢性的水样腹泻并导致脱水，低钾，胃酸缺乏，酸中毒，潮红和低血压（由血管舒张引起），高钙血症和高血糖综合征。这种综合症称为Verner-Morrison综合症（VMS），WDHA综合症（源于腹泻，低血钾，胃酸缺乏症）或胰腺霍乱综合征（PCS）。
临床表现
主要临床特征是长时间的水样腹泻（大便量> 750至1000 mL/天）以及低钾血症和脱水症状。一半的病人腹泻相对稳定，其余的病人交替出现重度和中度腹泻。三分之一的人在诊断前腹泻<1年，但有25%的人在诊断前出现腹泻5年或更久。 嗜睡，肌肉无力，恶心，呕吐和腹部绞痛是常见的症状。低钾血症和受损的葡萄糖耐受性发生在<50%的患者中。胃酸缺乏症也是一个症状，在腹泻发作期间，很少发生类似于类癌综合征的潮红。
诊断方法
除临床表现外，空腹血浆水平可确诊， CT扫描和生长抑素受体闪烁显像术可定位肿瘤，通常在转移时表现为转移性。
测试包括：血液化学测试；腹部CT扫描；腹部MRI；检查腹泻和电解质水平紊乱的原因；血液中的血管活性肠肽（VIP）水平。
治疗方法
治疗的首要目标是纠正脱水。通常通过静脉注射液体（静脉输液），以补充因腹泻而流失的液体。下一个目标是减缓腹泻，一些药物可以帮助控制腹泻，奥曲肽是人为形成的天然激素生长抑素的一种形式，它具有阻止VIP的作用。治愈的最佳方式是手术切除肿瘤，如果肿瘤尚未扩散到其他器官，则手术通常可以治愈该病。对于转移性疾病，肽受体放射性核素治疗（PRRT）可能非常有效。该治疗包括将放射性核素（L177或钇90）与生长抑素类似物（奥曲肽）连接。这是传递高剂量β射线杀死肿瘤的新颖方法。有些人似乎对称为卡培他滨和替莫唑胺的化学联合疗法有反应，但是没有报道说它可以完全治愈人们的舒血管肠肽瘤。
预后
预后取决于疾病的状况，通过手术可以治愈，如果尚未扩散，则预后良好。但是，如果肿瘤在诊断之前已经转移，则预后不良且无法治愈。
舒血管肠肽瘤与类癌
良性肿瘤和舒血管肠肽瘤之间的区别是良性肿瘤影响胃肠道（胃和肠），而舒血管肠肽瘤则影响胰腺。当血清素和其他激素过量产生时，会产生良性肿瘤。同时，舒血管肠肽瘤是一种与血管活性肠肽产生有关的疾病。另外，它是生长缓慢的神经内分泌癌，起源于神经内分泌细胞系统。这意味着它仅影响接受神经输入的激素释放细胞。它通常在回肠中肠（小肠的最后部分）中发现，并影响呼吸道，良性肿瘤和舒血管肠肽瘤具有共同的症状，都会腹泻，并且都可以通过手术程序进行治疗。
Life-threating diarrhea and acute renal failure secondary to pancreatic VIPoma treated by surgery
复制标题
手术治疗胰腺VIPoma继发的危及生命的腹泻和急性肾功能衰竭
发表时间：2019-08-15
影响指数：1.9
作者： María Lapena Rodríguez
期刊：Rev Esp Enferm Dig
A hormone-secreting neuroendocrine tumor should be suspected due to the clinical scenario of profuse watery diarrhea of more than one liter per day, dehydration, hypokalemia and metabolic acidosis. The difficult management is based on the low clinical suspicion due to the incidence and the rapid clinical deterioration in this case. A rapid hydroelectric replenishment and treatment with octreotide is crucial to avoid an acute renal failure, as in our patient. Pancreatic neuroendocrine tumors (PNET) account for less than 5% of all pancreatic tumors and can present as nonfunctioning and functioning PNETs. The second group includes the pancreatic VIPoma and its incidence is < 1 case/10,000,000 inhabitants/year. It originates in the non-β cells of the pancreatic islets that produce vasoactive intestinal peptide. They are usually isolated tumors, larger than 3 cm in diameter and are located in the tail of the pancreas in 70% of cases. Five per cent of these tumors form part of the multiple endocrine neoplasia syndrome type 1 (MEN1). The case presented here is a 46-year-old patient with a large isolated lesion, located in the body of the pancreas. These tumors produce the Verner-Morrison clinical syndrome, pancreatic cholera or watery diarrhea hypokaliemia achlorydria (WDHA). This syndrome is characterized by severe watery diarrhea with a loss of bicarbonate and potassium, which in turn gives rise to hypovolemia with hypokalemia and metabolic acidosis. It is associated with hypochlorhydria or achlorhydria in 75% of cases. Clinical history and VIP values higher than 200 pg/ml leads to a diagnosis. The main imaging techniques used for the diagnosis and follow-up of PNETs are ultrasound, CT, MRI or even OctreoScan®. Endoscopic ultrasound has a higher sensitivity than CT and MRI (92 and 93%, respectively) to visualize smaller tumors. In our case, CT and MRI allowed us to specify the tumor size and location. Endoscopic ultrasound was also performed, which allowed samples to be obtained via FNAP and helped to plan the surgical strategy. The study was completed with an OctreoScan®, whose sensitivity depends on the density of somatostatin receptors of the tumor cells where the radioisotope is fixed.
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