Neuroendocrine neoplasms


Editor-in-Chief: Debra L. Zynger, M.D.
Raul S. Gonzalez, M.D.

Last author update: 10 December 2020
Last staff update: 6 September 2022

Copyright: 2002-2023,, Inc.

PubMed Search: VIPoma pancreas

Raul S. Gonzalez, M.D.
Page views in 2022: 878
Page views in 2023 to date: 72
Cite this page: Gonzalez R. VIPoma. website. Accessed January 30th, 2023.
Definition / general
  • Well differentiated neuroendocrine tumor that secretes vasoactive intestinal peptide (VIP), leading to chronic watery diarrhea
Essential features
  • Functional well differentiated neuroendocrine tumor (WD-NET) that secretes vasoactive intestinal peptides
  • Histology is same as other well differentiated neuroendocrine tumors in pancreas
  • Clinical diarrhea can be severe, possibly fatal (may exceed 8 liters of stool in 24 hours) (South Dartmouth (MA):, Inc;2017 Jun 5)
ICD coding
  • ICD-10:
    • C7A.8 - other malignant neuroendocrine tumors
    • E16.8 - other specified disorders of pancreatic internal secretion
  • VIP stimulates intestinal secretion of water and electrolytes, causing VIPoma syndrome when excessive
  • Most cases are sporadic but about 5% occur in the setting of multiple endocrine neoplasia 1 syndrome (MEN1)
Clinical features
  • Imaging to detect pancreatic mass
  • Elevated plasma vasoactive intestinal levels
Radiology images

Images hosted on other servers:

Pancreatic tail mass

Liver metastasis

Prognostic factors
Case reports
  • Surgical resection of primary lesion, if possible
  • Hepatic metastases can be resected or embolized
  • Octreotide for metastatic disease
Gross description
  • Single or multiple lesions, most commonly in tail of pancreas
  • Firm, homogeneous, well circumscribed
Microscopic (histologic) description
  • Nests of monotonous low grade neuroendocrine cells with salt and pepper nuclei and ample amphophilic cytoplasm, as with any other well differentiated neuroendocrine tumor
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Nests and cords of cells

Positive stains
Electron microscopy images

AFIP images

Pancreatic VIPoma

Molecular / cytogenetics description
Sample pathology report
  • Pancreas and duodenum, Whipple procedure:
    • Well differentiated neuroendocrine tumor of pancreas (see comment and synoptic report)
    • Comment: Based on the patient's clinical symptoms, this neuroendocrine tumor is best considered a VIPoma.
Differential diagnosis
Board review style question #1
What is the most common symptom of a VIPoma?

  1. Abdominal pain
  2. Acromegaly
  3. Diarrhea
  4. Necrotizing migratory erythema
  5. Weakness
Board review style answer #1
C. Diarrhea

Comment here

Reference: VIPoma
Board review style question #2

Where are VIPomas most likely to arise?

  1. Body of the pancreas
  2. Duodenum
  3. Head of the pancreas
  4. Ileum
  5. Tail of the pancreas
Board review style answer #2
E. Tail of the pancreas

Comment here

Reference: VIPoma
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