Neuroendocrine neoplasms

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

Topic Completed: 26 July 2019

Minor changes: 7 November 2019

Copyright: (c) 2002-2019,, Inc.

PubMed Search: VIPoma[TI] pancreas

Raul S. Gonzalez, M.D.
Page views in 2019: 579
Page views in 2020 to date: 414
Cite this page: Gonzalez R. VIPoma. website. Accessed September 20th, 2020.
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
  • ICD-10: 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
Gross images

Images hosted on other servers:

Pancreatic head tumor

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
The most common symptom of a VIPoma is

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

Reference: Pancreas - VIPoma

Comment here
Board review style question #2
VIPomas are most likely to arise in the

  1. Body of the pancreas
  2. Duodenum
  3. Head of the pancreas
  4. Ileum
  5. Tail of the pancreas

Board review answer #2
E. Tail of the pancreas

Reference: Pancreas - VIPoma

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