血管活性肠肽肿瘤

Naveed M. Natanzi, M. Amini, D. Yamini, S. Nielsen, R. Ram
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引用次数: 8

摘要

背景和目的。血管活性肠肽肿瘤是一种罕见的神经内分泌肿瘤,通过血管活性肠肽介导的循环amp依赖机制,通过肠粘膜分泌大量电解质和水导致大量水样腹泻。由于水和电解质的流失而产生的酸碱失衡会导致严重脱水和潜在的肾衰竭,如果不及时治疗,最终会导致死亡。本文旨在回顾本病的临床、组织学、放射学和诊断特点,以及治疗此病的治疗方法。方法。使用MEDLINE、Pubmed和Cochrane数据库进行文献回顾,使用MeSH术语收集数据,包括血管活性肠肽、VIPoma和WDHA。结果与结论。血管活性肠肽肿瘤是一种罕见的肿瘤,通过胃肠道分泌水和电解质,发病率和死亡率高。这种肿瘤的非特异性临床表现可能给诊断带来挑战,因为这些肿瘤很容易被误诊为其他疾病,从泻药过量到存在类癌分泌性肿瘤。尽管如此,一些影像学和实验室研究可以促进对VIPoma的正确评估和诊断。在正确诊断后,根据转移的存在和程度,通过药物或手术方式治疗VIPomas。
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Vasoactive Intestinal Peptide Tumor
Background and aim. Vasoactive intestinal peptide tumor is a rare neuroendocrine neoplasm which causes voluminous watery diarrhea via hypersecretion of electrolytes and water from the intestinal mucosa through a vasoactive intestinal peptide-mediated, cyclic AMP-dependent mechanism. The acid base imbalance generated by the loss of water and electrolytes leads to severe dehydration and potential renal failure, which can ultimately result in death if left untreated. This paper aims to review the clinical, histological, radiological, and diagnostic features of this disease as well as the therapeutic modalities in treating this condition. Methods. A review of literature was performed using MEDLINE, Pubmed, and Cochrane databases in collection of data using MeSH terms including vasoactive intestinal peptide, VIPoma, and WDHA. Results and conclusion. Vasoactive intestinal peptide tumor is a rare neoplasm associated with significant morbidity and mortality through secretion of water and electrolytes in the gastrointestinal tract. The nonspecific clinical presentation of this neoplasm can pose diagnostic challenges, as these tumors can be easily misdiagnosed as other conditions, ranging from laxative overdose to the presence of a carcinoid secreting tumor. Nevertheless, a number of imaging and laboratory studies can facilitate the correct evaluation and diagnosis of VIPoma. Following proper diagnosis, VIPomas are treated by either medical or surgical modalities depending on the existence and extent of metastasis.
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