Department of Hepato-Gastroenterology and Digestive Oncology, Robert-Debré Hospital and Reims-Champagne-Ardenne University, Reims, France.
Department of Digestive Oncology, ENETS Centre of Excellence, Hospices Civils de Lyon, Lyon, France.
Pancreatology. 2021 Dec;21(8):1531-1539. doi: 10.1016/j.pan.2021.08.001. Epub 2021 Aug 5.
Vasoactive intestinal peptide-secreting tumor (VIPoma) is a very rare, life-threatening, functioning pancreatic neuroendocrine tumor (pNET). The efficacy of antitumor therapies against functioning symptoms and tumor burden have been poorly described in VIPoma.
Describe the impact of treatments on the secretory syndrome, tumor burden and survival in patients with VIPoma.
We retrospectively reviewed the records of patients with VIPoma treated in seven French expert centers between 1990 and 2016. Diagnostic of VIPoma was reassessed using strict criteria. We evaluated the antisecretory efficacy (>50 % decrease of daily bowel movements), and antitumor efficacy (RECIST 1.1) of all treatments received.
Twenty-two patients were included. pNETs were mostly metastatic (77 %) and classified as grade 2 (83 %). Median follow-up was 78.2 months. Surgical excision of nonmetastatic VIPoma effectively controlled the secretory syndrome. Although 4/5 patients had metastatic recurrences, all patients were alive after median post-operative follow-up of 171 months. Among the 87 treatments received for metastatic VIPoma, curative-intent surgery (n = 14), somatostatin analogs alone (n = 11), chemotherapy (n = 23), transarterial liver embolization (TALE) (n = 14), everolimus (n = 10) and sunitinib (n = 7) achieved, respectively, 100 %, 67 %, 83 %, 50 %, 20 % and 100 % antisecretory efficacy. The 5-year OS rate was 63.6 %, with pejorative impact of higher Ki-67 index (P = 0.045) and higher plasma VIP concentration (P = 0.025).
Surgical resection of localized VIPoma is effective but rarely curative. For metastatic VIPoma, curative-intent surgery, chemotherapy and sunitinib are the therapeutic options that best combined antitumor and antisecretory efficacies.
血管活性肠肽分泌肿瘤（VIPoma）是一种非常罕见、危及生命的功能性胰腺神经内分泌肿瘤（pNET）。针对功能性症状和肿瘤负荷的抗肿瘤治疗效果在 VIPoma 中描述得很差。
描述治疗对 VIPoma 患者的分泌综合征、肿瘤负荷和生存的影响。
我们回顾性分析了 1990 年至 2016 年间在法国 7 个专家中心治疗的 VIPoma 患者的记录。使用严格的标准重新评估 VIPoma 的诊断。我们评估了所有治疗的抗分泌疗效（每日排便量减少 50%以上）和抗肿瘤疗效（RECIST 1.1）。
共纳入 22 例患者。pNET 主要为转移性（77%），分级为 2 级（83%）。中位随访时间为 78.2 个月。非转移性 VIPoma 的手术切除可有效控制分泌综合征。尽管 4/5 例患者发生转移性复发，但所有患者在术后中位随访 171 个月后仍存活。在 87 例转移性 VIPoma 的治疗中，根治性手术（n=14）、生长抑素类似物单独治疗（n=11）、化疗（n=23）、经肝动脉栓塞（TALE）（n=14）、依维莫司（n=10）和舒尼替尼（n=7）的抗分泌疗效分别达到 100%、67%、83%、50%、20%和 100%。5 年 OS 率为 63.6%，Ki-67 指数较高（P=0.045）和血浆 VIP 浓度较高（P=0.025）与预后较差相关。
局限性 VIPoma 的手术切除是有效的，但很少能治愈。对于转移性 VIPoma，根治性手术、化疗和舒尼替尼是结合抗肿瘤和抗分泌疗效的最佳治疗选择。
