Vasoactive Intestinal Polypeptide Secreting Tumor - VIPoma

Vasoactive intestinal polypeptide secreting tumors (VIPomas) are very rare pancreatic neuroendocrine tumors (PNETs) associated with profuse diarrhea. They are usually single tumors and metastatic at diagnosis. Generally, laboratory testing includes an electrolyte panel and vasoactive intestinal peptide test.

Tabs Content

Clinical Overview

Diagnosis

Indications for Testing

Chronic, profuse, secretory diarrhea

Criteria for Diagnosis

Documented elevated (>200 pg/mL) vasoactive intestinal polypeptide (VIP) concentration (National Cancer Institute [NCI], 2018) in patient with large-volume secretory diarrhea (Vinik, North American Neuroendocrine Tumor Society [NANETS], 2010)
- VIP level >75 pg/mL highly suggestive
Large-volume diarrhea (Kulke, NANETS, 2010)
- 70-80% of patients with VIPoma have more than 3L/day
- Virtually all patients with VIPoma have >700mL/day
Hypokalemia
Achlorhydria

Laboratory Testing

Electrolytes
- Hypokalemia in 100% of patients
VIP level

Histology

Definitive diagnosis requires biopsy and pathologist examination
- Useful immunohistochemical stains may include chromogranin A, synaptophysin, and cytokeratin
- For detailed descriptions, including recommended tests, refer to ARUP’s Immunohistochemistry Stain Offerings

Imaging Studies

Computed tomography (CT)/magnetic resonance imaging (MRI) or endoscopic ultrasound
Somatostatin-receptor scintigraphy

Differential Diagnosis

Gastroenteritis (chronic or relapsing)
Zollinger-Ellison syndrome (gastrinoma)
Carcinoid tumor
Villous adenoma
Inflammatory bowel disease
Malabsorption
Laxative abuse
Factitious diarrhea
Pancreatic cancer
Somatostatinoma

Monitoring

Plasma chromogranin – often used to monitor functional and nonfunctional PNETs after resection (Vinik, NANETS, 2010)

Background

Epidemiology

Incidence – 1/10 million (rare cause of functioning PNETs)
Age – median onset 40s
Sex – M:F, equal
Occurrence – sporadic, although some VIPomas occur in association with multiple endocrine neoplasia type 1 (MEN1)

Risk Factors

Genetic – ~5% associated with MEN1 (Jensen, 2012)

Pathophysiology

Most tumors occur in the body and tail of the pancreas and are unifocal (other locations include adrenal, paraganglia)
Symptoms result from the secretion of the peptide hormone VIP, an amino acid polypeptide that is a potent stimulator of intestinal secretion and inhibitor of gastric acid secretion
Other tumors can produce VIP – ganglioneuroblastoma, bronchogenic carcinoma, pheochromocytoma, and medullary thyroid carcinoma

Clinical Presentation

Verner-Morrison syndrome (pancreatic cholera)
- Chronic watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome)
- Profuse diarrhea (may exceed 6-8L/day) causes dehydration, metabolic acidosis, and renal failure

ARUP Lab Tests

Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

Electrolyte Panel 0020410
Method: Quantitative Ion-Selective Electrode/Enzymatic

Recommended Use

Initial testing in evaluation of diarrhea

Vasoactive Intestinal Peptide 0099435
Method: Quantitative Radioimmunoassay

Recommended Use

Aid in the diagnosis of VIPoma

Chromogranin A 0080469
Method: Quantitative Enzyme Immunoassay

Recommended Use

Aid in monitoring; not recommended for diagnosis of carcinoid tumors

May be useful in monitoring nonsecretory sympathetic and parasympathetic neuroendocrine tumors

Chromogranin A by Immunohistochemistry 2003830
Method: Immunohistochemistry

Recommended Use

Aid in histologic identification of neuroendocrine tissue

Stained and returned to client pathologist; consultation available if needed

Synaptophysin by Immunohistochemistry 2004139
Method: Immunohistochemistry

Recommended Use

Aid in histologic identification of neuroendocrine tissue

Stained and returned to client pathologist; consultation available if needed

Pan Cytokeratin (AE1,3) by Immunohistochemistry 2003433
Method: Immunohistochemistry

Recommended Use

Aid in histologic diagnosis of VIPoma

Stained and returned to client pathologist; consultation available if needed

Ki-67 with Interpretation by Immunohistochemistry 2007182
Method: Immunohistochemistry

Recommended Use

Aid in grading of neuroendocrine tumors

Determine mitotic rate of tissue

Stained and resulted by ARUP

Neuron Specific Enolase, Polyclonal (NSE P) by Immunohistochemistry 2004052
Method: Immunohistochemistry

Recommended Use

Aid in histologic identification of neural and neuroendocrine tissue

Stained and returned to client pathologist; consultation available if needed

Medical Experts

Straseski, Joely A., PhD, MS, MT(ASCP), DABCC, Medical Director, Endocrinology and Automated Core Laboratory at ARUP Laboratories; Associate Professor of Clinical Pathology, University of Utah

References

Guidelines

NCCN Clinical Practice Guidelines in Oncology, Neuroendocrine and Adrenal Tumors. Version 4.2018. National Comprehensive Cancer Network. Fort Washington, PA [Updated: May 2018; Accessed: Jul 2018]
O'Toole D, Grossman A, Gross D, Fave GD, Barkmanova J, O'Connor J, Pape U, Plöckinger U, Mallorca Consensus Conference participants, European Neuroendocrine Tumor Society. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: biochemical markers. Neuroendocrinology. 2009; 90(2): 194-202. PubMed
Vinik AI, Woltering EA, Warner RR, Caplin M, O'Dorisio TM, Wiseman GA, Coppola D, Go VL, North American Neuroendocrine Tumor Society (NANETS). NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. Pancreas. 2010; 39(6): 713-34. PubMed
Oberg K, Knigge U, Kwekkeboom D, Perren A, ESMO Guidelines Working Group. Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012; 23 Suppl 7: vii124-30. PubMed
Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, Scoazec J, Salazar R, Sauvanet A, Kianmanesh R, Barcelona Consensus Conference participants. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology. 2012; 95(2): 98-119. PubMed
Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ) – Health Professional Version. National Cancer Institute. [Updated: Feb 2018; Accessed: Aug 2018]
Oberg K, Couvelard A, Fave GD, Gross D, Grossman A, Jensen RT, Pape U, Perren A, Rindi G, Ruszniewski P, Scoazec J, Welin S, Wiedenmann B, Ferone D, all other Antibes Consensus Conference participants. ENETS Consensus Guidelines for Standard of Care in Neuroendocrine Tumours: Biochemical Markers. Neuroendocrinology. 2017; PubMed
Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, Kos-Kudla B, Kwekkeboom D, Rindi G, Klöppel G, Reed N, Kianmanesh R, Jensen RT, Vienna Consensus Conference participants. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology. 2016; 103(2): 153-71. PubMed
Kulke MH, Anthony LB, Bushnell DL, de Herder WW, Goldsmith SJ, Klimstra DS, Marx SJ, Pasieka JL, Pommier RF, Yao JC, Jensen RT, North American Neuroendocrine Tumor Society (NANETS). NANETS treatment guidelines: well-differentiated neuroendocrine tumors of the stomach and pancreas. Pancreas. 2010; 39(6): 735-52. PubMed

General References

Batcher E, Madaj P, Gianoukakis AG. Pancreatic neuroendocrine tumors. Endocr Res. 2011; 36(1): 35-43. PubMed

Kapoor R, Moseley RH, Kapoor JR, Crapo LM, Saint S. Clinical problem-solving. Needle in a haystack. N Engl J Med. 2009; 360(6): 616-21. PubMed

Oberg K. Pancreatic endocrine tumors. Semin Oncol. 2010; 37(6): 594-618. PubMed

Educational Videos

Content Review:

September 2017

Last Update: February 2019

Vasoactive Intestinal Polypeptide Secreting Tumor - VIPoma

Diagnosis

Indications for Testing

Criteria for Diagnosis

Laboratory Testing

Histology

Imaging Studies

Differential Diagnosis

Monitoring

Background

Epidemiology

Risk Factors

Pathophysiology

Clinical Presentation

ARUP Lab Tests

Medical Experts

References

Guidelines

General References

ARUP Laboratories

ARUP Websites

ARUP Consult


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	Vasoactive Intestinal Polypeptide Secreting Tumor - VIPoma. ARUP Consult^©. Retrieved February 20, 2019, from: https://arupconsult.com/content/vasoactive-intestinal-polypeptide-secreting-tumor