Schizas Dimitrios, Mastoraki Aikaterini, Bagias George, Patras Raphael, Moris Dimitrios, Lazaridis Ioannis I, Arkadopoulos Nikolaos, Felekouras Evangelos
1st Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
J BUON. 2019 Mar-Apr;24(2):415-423.
Vasoactive intestinal peptide (VIP) secreting tumor (VIPoma) constitutes a rare functional neuroendocrine tumor that most often originates from pancreatic islet cells and presents as a sporadic, solitary neoplasm of the pancreas. The purpose of this study was to systematically review the literature of pancreatic VIPomas and report clinicopathologic data and treatment modalities for this rare entity.
A systematic literature search was performed. The reviewed clinical series and case reports were included if they reported surgical treatment and also analyzed oncological outcomes on individual patients. Data extraction was performed using a standard registry pro-forma.
The search resulted in 53 case reports and 2 case series including 65 patients in total. Median age reported was 54 years. The predominant pancreatic location was the pancreatic tail. The most common clinical symptom was watery diarrhea. Serum VIP levels were remarkably elevated in all patients. Distal pancreatectomy with or without splenectomy was the most commonly applied surgical procedure. Overall survival associated with pancreatic VIPoma was 67.7%, recurrence rate 40.4% and relevant median disease-free interval was 16 months.
VIPomas are functional tumors that secrete excessive amounts of VIP. Clinically, production of VIP causes refractory watery diarrhea, hypokalemia and achlorydria. As far as diagnosis is concerned, elevated VIP plasma levels are required. Moreover, the majority of VIPomas are malignant or have already metastasized on diagnosis. Despite recent research on the therapeutic strategies against pancreatic VIPoma, surgical resection appears as the only potentially curative approach.
血管活性肠肽（VIP）分泌瘤（VIPoma）是一种罕见的功能性神经内分泌肿瘤，最常起源于胰岛细胞，表现为胰腺的散发性孤立性肿瘤。本研究的目的是系统回顾胰腺VIPoma的文献，并报告这种罕见疾病的临床病理数据和治疗方式。
进行了系统的文献检索。纳入的综述临床系列和病例报告需报告手术治疗情况，并分析个体患者的肿瘤学结局。使用标准注册表格式进行数据提取。
检索结果包括53篇病例报告和2个病例系列，共65例患者。报告的中位年龄为54岁。胰腺的主要位置是胰尾。最常见的临床症状是水样腹泻。所有患者的血清VIP水平均显著升高。行或不行脾切除术的远端胰腺切除术是最常用的手术方法。胰腺VIPoma患者的总生存率为67.7%，复发率为40.4%，相关的无病间期中位数为16个月。
VIPoma是分泌过量VIP的功能性肿瘤。临床上，VIP的产生会导致难治性水样腹泻、低钾血症和无胃酸。就诊断而言，需要检测血浆VIP水平升高。此外，大多数VIPoma在诊断时即为恶性或已发生转移。尽管最近对胰腺VIPoma的治疗策略进行了研究，但手术切除似乎是唯一可能治愈的方法。
