Pancreas
Neuroendocrine neoplasms
VIPoma
Author: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 10 December 2020
Last staff update: 6 September 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: VIPoma pancreas
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy images | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Gonzalez R. VIPoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasvipoma.html. Accessed April 26th, 2024.
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): MDText.com, Inc;2017 Jun 5)
Terminology
- VIPoma syndrome is also termed Verner-Morrison syndrome and WDHA syndrome (watery diarrhea, hypokalemia, achlorhydria) (Rom J Morphol Embryol 2017;58:371)
ICD coding
Epidemiology
- Incidence is roughly 1 per 10 million persons per year (Surg Clin North Am 1987;67:379)
- Mean age 51 years
Sites
- 90% of VIPomas arise from pancreas (Am J Med 1987;82:37)
- Most common pancreatic location is tail (Surgery 1998;124:1050)
Pathophysiology
- VIP stimulates intestinal secretion of water and electrolytes, causing VIPoma syndrome when excessive
Etiology
- Most cases are sporadic but about 5% occur in the setting of multiple endocrine neoplasia 1 syndrome (MEN1)
Clinical features
- Chronic secretory diarrhea hypokalemia, achlorhydria, flushing, hypercalcemia (Hepatogastroenterology 2005;52:1259)
Diagnosis
- Imaging to detect pancreatic mass
- Elevated plasma vasoactive intestinal levels
Radiology images
Images hosted on other servers:
Pancreatic tail mass
Liver metastasis
Prognostic factors
- Often metastatic at time of diagnosis (Surgery 1998;124:1050)
- Median survival is roughly 6 years (Pancreas 2019;48:934)
- Poor prognostic factors: age < 40 years or > 60, size > 4 cm, poor clinical management of water, electrolyte and acid base profiles, metastatic disease, unresectability (J Clin Case Rep 2012,2:15)
Case reports
- 13 year old girl with multiple endocrine neoplasia 1 syndrome and pancreas mass (Indian J Endocrinol Metab 2013;17:S215)
- 46 year old woman with 18 cm tumor (World J Surg Oncol 2008;6:80)
- 47 year old man with sporadic tumor (Hematol Oncol Stem Cell Ther 2014;7:109)
- 74 year old man with diarrhea, weight loss and liver metastasis (Case Rep Gastroenterol 2019;13:225)
Treatment
- 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
- VIP (though this does not establish the diagnosis)
- Synaptophysin, chromogranin
- PDX1, INSM1 (Am J Surg Pathol 2012;36:737)
Negative stains
Electron microscopy images
AFIP images
Pancreatic VIPoma
Molecular / cytogenetics description
- Overexpression of CXCR4, down regulation of MSH2 in one case (World J Surg Oncol 2012;10:264)
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
- Nongastrin secreting pancreatic neuroendocrine tumor:
- No histopathologic differences; must be determined on clinical grounds
Board review style question #1
What is the most common symptom of a VIPoma?
- Abdominal pain
- Acromegaly
- Diarrhea
- Necrotizing migratory erythema
- Weakness
Board review style answer #1
Board review style question #2
Where are VIPomas most likely to arise?
- Body of the pancreas
- Duodenum
- Head of the pancreas
- Ileum
- Tail of the pancreas
Board review style answer #2
