VIP-Mediated Syndromes: Contrasting Presentations and Multidisciplinary Management of Two Rare Neuroendocrine Tumors

ABSTRACT Vasoactive intestinal peptide (VIP)-secreting tumors are rare neuroendocrine neoplasms that cause severe secretory diarrhea and electrolyte abnormalities. We describe 2 atypical cases requiring multidisciplinary care. One patient had a VIP-secreting pheochromocytoma with elevated VIP and metanephrine levels; symptoms improved after adrenalectomy. The second had a metastatic small intestinal neuroendocrine tumor with persistent VIP-mediated diarrhea despite debulking and somatostatin analog therapy, complicated by anal fissures, trans-sphincteric perianal fistula, and neuropathic pelvic pain. These cases highlight morbidity and potential complications associated with extrapancreatic VIP hypersecretion and the value of coordinated multidisciplinary management to improve outcomes and quality of life.

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Publication Details

Journal
ACG Case Reports Journal
Published
2026-10-28
DOI
https://doi.org/10.14309/crj.0000000000002382
Primary Topic
Neuroendocrine Tumor Research Advances
Type
article
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article

VIP-Mediated Syndromes: Contrasting Presentations and Multidisciplinary Management of Two Rare Neuroendocrine Tumors

Imran L. Balouch, Nikhil R. Kalva, Fatima Ahson, Yusuf Kagzi
1 links
ACG Case Reports Journal
Neuroendocrine Tumor Research Advances
article

VIP-Mediated Syndromes: Contrasting Presentations and Multidisciplinary Management of Two Rare Neuroendocrine Tumors

Imran L. Balouch, Nikhil R. Kalva, Fatima Ahson, Yusuf Kagzi
article en

Abstract

ABSTRACT Vasoactive intestinal peptide (VIP)-secreting tumors are rare neuroendocrine neoplasms that cause severe secretory diarrhea and electrolyte abnormalities. We describe 2 atypical cases requiring multidisciplinary care. One patient had a VIP-secreting pheochromocytoma with elevated VIP and metanephrine levels; symptoms improved after adrenalectomy. The second had a metastatic small intestinal neuroendocrine tumor with persistent VIP-mediated diarrhea despite debulking and somatostatin analog therapy, complicated by anal fissures, trans-sphincteric perianal fistula, and neuropathic pelvic pain. These cases highlight morbidity and potential complications associated with extrapancreatic VIP hypersecretion and the value of coordinated multidisciplinary management to improve outcomes and quality of life.

ACG Case Reports JournalVol. 13(11)
Peoria campus of the University of Illinois System (US)
Good health and well-being
Openalex Percentile: Top 12%
Neuroendocrine Tumor Research Advances
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