VIPoma

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 22 Apr 2025

Last updated: 26 Mar 2025

VIPomas are rare functional neuroendocrine tumors typically originating within the pancreas.

VIPoma is characterized by profuse watery diarrhea, hypokalemia, metabolic acidosis, and hypochlorhydria or achlorhydria, in the presence of elevated serum vasoactive intestinal peptide (VIP).

The clinical syndrome associated with VIPomas is sometimes referred to as WDHA syndrome (watery diarrhea, hypokalemia, and achlorhydria), Verner-Morrison syndrome, or pancreatic cholera.

VIPomas are highly malignant. Patients usually present with regional lymph node or liver involvement at diagnosis.

Surgical resection is the standard treatment.

Symptoms are managed by hydration, electrolyte repletion, somatostatin analogs, and/or surgical debulking if metastatic.

Definition

VIPomas are rare functional neuroendocrine tumors, highly malignant, often presenting with regional lymph node or liver involvement at diagnosis. They are usually solitary tumors, with over 90% originating in the pancreas. The remaining cases arise in extrapancreatic locations, such as the retroperitoneum or thorax.[1]

VIPomas secrete VIP, causing a clinical syndrome characterized by profuse watery diarrhea, hypokalemia, metabolic acidosis, and hypochlorhydria or achlorhydria.

History and exam

Key diagnostic factors

profuse watery diarrhea

Full details

Other diagnostic factors

age around 50 years
headache
weight loss
poor skin turgor
dry mucus membranes
muscle weakness
muscle cramps
gastrointestinal symptoms
hyperglycemia symptoms
flushing
hepatomegaly
history of multiple endocrine neoplasia type 1 (MEN1)

Full details

Risk factors

multiple endocrine neoplasia type 1 (MEN1)
age around 50 years

Full details

Diagnostic tests

1st tests to order

serum potassium
serum bicarbonate
serum calcium
serum glucose
vasoactive intestinal peptide (VIP) radioimmunoassay
liver function tests

Full details

Tests to consider

arterial blood gas analysis
gastric pH monitoring
chromogranin A
pancreatic polypeptide
contrast-enhanced CT scan of abdomen
contrast-enhanced MRI of abdomen
somatostatin receptor PET-CT/MRI
somatostatin receptor scintigraphy
endoscopic ultrasound
operative exploration
intraoperative ultrasound
biopsy

Full details

Treatment algorithm

ACUTE

initial presentation of symptomatic patient

ONGOING

localized disease

metastatic disease: surgical candidate

metastatic disease: nonsurgical candidate

refractory disease

Contributors

Authors

Harish Lavu, MD, FACS

Professor of Surgery

Sidney Kimmel Medical College

Thomas Jefferson University

Philadelphia

Disclosures

HL declares that he has no competing interests.

Charles J. Yeo, MD, FACS

The Samuel D. Gross Professor and Chair

Department of Surgery

Sidney Kimmel Medical College

Thomas Jefferson University

Philadelphia

Disclosures

CJY declares that he has no competing interests.

Peer reviewers

Alexandria Phan, MD

Professor of Medicine

Froedtert Hospital and the Medical College of Wisconsin

Milwaukee

Disclosures

AP declares that she has no competing interests.

Jonathan Strosberg, MD

Professor

Moffitt Cancer Center

Tampa

Disclosures

JS has consulted for Novartis and serves on the Speakers’ Bureau for Ipsen.

Jaime Ruiz-Tovar, MD

Fellow of the Spanish Association of Surgeons (AEC)

Department of Surgery

University Hospital Ramon y Cajal

Madrid

Spain

Disclosures

JRT declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

Hofland J, Falconi M, Christ E, et al. European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes. J Neuroendocrinol. 2023 Aug;35(8):e13318.Full text Abstract

Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.Full text

Del Rivero J, Perez K, Kennedy EB, et al. Systemic therapy for tumor control in metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline. J Clin Oncol. 2023 Nov 10;41(32):5049-67.Full text Abstract

Reference articles

A full list of sources referenced in this topic is available here.

Use of this content is subject to our disclaimer