Prognosis is dependent on tumor stage and the presence and severity of metastasis at diagnosis. Resection is associated with superior survival.[1]Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res. 1998 Dec;17(4):389-400.
http://www.ncbi.nlm.nih.gov/pubmed/10089056?tool=bestpractice.com
[65]Roland CL, Bian A, Mansour JC, et al. Survival impact of malignant pancreatic neuroendocrine and islet cell neoplasm phenotypes. J Surg Oncol. 2012 May;105(6):595-600.
http://www.ncbi.nlm.nih.gov/pubmed/22006521?tool=bestpractice.com
However, neuroendocrine tumor survival data are limited because of low incidence.[66]van der Zwan JM, Trama A, Otter R, et al. Rare neuroendocrine tumours: results of the surveillance of rare cancers in Europe project. Eur J Cancer. 2013 Jul;49(11):2565-78.
http://www.ncbi.nlm.nih.gov/pubmed/23541566?tool=bestpractice.com
VIPomas are slow-growing tumors, therefore survival outcomes are relatively good, even for patients with metastatic disease. Based on US Surveillance, Epidemiology, and End Results (SEER) data (2009-2016), the median overall survival for pancreatic neuroendocrine tumors, including VIPoma, was 85 months.[7]Sonbol MB, Mazza GL, Mi L, et al. Survival and incidence patterns of pancreatic neuroendocrine tumors over the last 2 decades: a SEER database analysis. Oncologist. 2022 Jul 5;27(7):573-8.
https://academic.oup.com/oncolo/article/27/7/573/6555099
http://www.ncbi.nlm.nih.gov/pubmed/35348774?tool=bestpractice.com
Localized disease
Complete surgical extirpation of VIPoma results in a >90% 5-year survival.[1]Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res. 1998 Dec;17(4):389-400.
http://www.ncbi.nlm.nih.gov/pubmed/10089056?tool=bestpractice.com
However, <50% of patients have localized disease at the time of diagnosis.[42]Perry RR, Vinik AI. Clinical review 72: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab. 1995 Aug;80(8):2273-8.
http://www.ncbi.nlm.nih.gov/pubmed/7629220?tool=bestpractice.com
Metastatic disease
The majority of patients with VIPoma will have distant metastasis at the time of diagnosis (>50%).[42]Perry RR, Vinik AI. Clinical review 72: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab. 1995 Aug;80(8):2273-8.
http://www.ncbi.nlm.nih.gov/pubmed/7629220?tool=bestpractice.com
These tumors are slow-growing and characteristically have scant mitosis. The only method by which malignancy is diagnosed is by identification of tumor cells in lymph nodes or distant sites. In one retrospective study of 241 cases for VIPoma, the 5-year survival for patients with metastatic disease exceeded 60%.[1]Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res. 1998 Dec;17(4):389-400.
http://www.ncbi.nlm.nih.gov/pubmed/10089056?tool=bestpractice.com