eMedicine Specialties > Endocrinology > Multiple Endocrine Disease and Miscellaneous Endocrine Disease

VIPomas

Daniel S Tung, MD, Fellow in Endocrinology, Department of Internal Medicine, Baylor College of Medicine
Sai-Ching Jim Yeung, MD, PhD, FACP, Deputy Section Chief of Emergency Care, Assistant Professor, Department of General Internal Medicine, Ambulatory Treatment and Emergency Care, University of Texas MD Anderson Cancer Center; Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine

Updated: Dec 18, 2008

Introduction

Background

The symptoms of VIPoma were described in 1958, when Verner and Morrison reported on 2 patients with a syndrome of watery diarrhea, hypokalemia, and achlorhydria (WDHA).¹ These patients eventually succumbed to the condition as a result of dehydration and renal failure, despite attempted intravenous hydration. In 1970, Said and Mutt extracted the putative hormone causing WDHA from pig gut tissue.² In 1973, Bloom causally linked this hormone to WDHA, in a report on 6 patients with watery diarrhea resulting from pancreatic tumors associated with raised plasma levels of this hormone. The extirpated tumors from these cases were found to contain large amounts of what is now known as vasoactive intestinal polypeptide (VIP).

Pathophysiology

Found mainly in the pancreas, VIPomas are neuroendocrine tumors that secrete vasoactive intestinal polypeptide (VIP) autonomously.

VIP has a molecular weight of 3381, consists of 28 amino acids, and belongs to the secretin-glucagon family. The VIP gene is located on chromosome 6. VIP is normally expressed in the central nervous system and in the neurons of the gastrointestinal, respiratory, and urogenital tracts, where it functions as a neurotransmitter. VIP regulates the synthesis, secretion, and action of other neuroendocrine hormones; it also regulates cytokines and chemokines. Deficiency of VIP leads to developmental and behavioral abnormalities, including impaired circadian rhythms, in animal models.³ Overexpression of VIP causes diarrhea and cancer, and overexpression of VIP receptors promotes cancerous growth. In the gastrointestinal tract, VIP is largely responsible for the relaxation of vascular and nonvascular smooth muscle and for water and electrolyte secretion. VIP is released in response to gut distension by food.

VIP is a potent stimulator of gut cyclic adenosine monophosphate (cAMP) production, which leads to massive secretion of water and electrolytes (mainly potassium). VIP resembles secretin, which stimulates the secretion of alkaline pancreatic juices. In the stomach, VIP inhibits histamine- and pentagastrin-stimulated acid secretion. Like glucagon, VIP stimulates lipolysis and glycogenolysis and has an inotropic effect on the myocardium. It also has anti-inflammatory properties and modulates the immune system.

VIPomas arise from the pancreas in 90% of cases, but they may also be found in periganglionic tissue or at other sites, including the colon, bronchus, adrenal glands, and liver, especially in children.⁴ These tumors are almost always solitary, with less than 5% of cases being multicentric. VIPomas are usually greater than 3 cm at the time of diagnosis and are found primarily in the body and tail of the pancreas.

Approximately 60-80% of VIPomas are malignant and have metastasized at the time of diagnosis. Metastasis occurs most frequently in the liver, but it may also occur in the lymph nodes, lung, or kidneys.⁵ Approximately 5% of VIPomas are associated with multiple endocrine neoplasia type 1 (MEN 1) syndrome. Conversely, 17% of patients with MEN 1 develop VIPomas at some stage of their disease. Approximately 10% of neuroendocrine tumors of the gastrointestinal tract (except carcinoids) are VIPomas.

Frequency

International

The incidence of new cases of VIPoma is 0.05-0.5 per million people per year.

Mortality/Morbidity

• Morbidity from untreated WDHA syndrome is associated with long-standing dehydration and with electrolyte and acid-base disturbances, which may cause chronic renal failure.⁶
• Death results from renal failure or cardiac arrest caused by volume depletion, hypokalemia, and severe acid-base disturbances. 

Sex

• The male-to-female ratio for VIPoma is approximately 1:1.   

Age

• Peak incidence occurs in the fifth decade of life, but VIPomas may occur in any age group, including in young children and elderly persons.

Clinical

History

• The onset of VIPoma is insidious.
• The dominant symptom is profuse diarrhea despite fasting; this symptom may persist for years before the diagnosis is established. Diarrhea may be episodic initially, but it becomes continuous as the tumor progresses. Stool volumes are typically profound, with volumes greater than 3 L per day in 70% of cases. The stool is typically odorless and tea-colored, without blood or mucus.
• The loss of water, sodium, and chloride may lead to volume depletion, dehydration, and exhaustion among patients who are unable to replace fluid and electrolyte losses. Weight loss and even renal failure have been reported in some patients. 
• Excretion of large amounts of potassium and bicarbonate in the stool causes hypokalemia and non–anion gap acidosis. Hypokalemia may present as muscle cramps and/or weakness.
• Abdominal discomfort or bloating has been reported. 
• Facial flushing involved one third of patients from a 31-case series in China.⁷ Other studies have also reported facial flushing, but without a reported frequency.  
• One reported case in China involved periodic backache and a rash involving the chest, back, and upper limb. These 2 symptoms occurred before or after the diarrhea, worsened over 6 years, and resolved after surgical resection.

Physical

• Volume depletion may lead to tachycardia, decreased skin turgor, and documented weight loss.
• Because of marked fecal loss of potassium, patients may have muscle weakness on examination.
• Patients may present with a mildly distended abdomen.
• Hepatomegaly may be detected if liver metastasis has occurred. 
• Facial flushing may be seen because of the vasodilatory effects of vasoactive intestinal polypeptide.
• An electrocardiogram may reveal QRS widening and T-wave flattening if hypokalemia is severe.

Causes

Point mutations on chromosome 11 of the MEN1 gene have been identified in cases where VIPomas are part of MEN 1 syndrome and, to a lesser extent, in sporadic tumors.

Differential Diagnoses

Other Problems to Be Considered

All conditions with diarrhea
Ganglioneuroblastoma (similar symptoms mainly in children)
Infectious diseases of the intestines
Laxative abuse
Villous adenoma of the rectum

Workup

Laboratory Studies

• A diagnosis of VIPoma is made when watery diarrhea, hypokalemia, and achlorhydria are present in the setting of elevated serum vasoactive intestinal polypeptide (VIP) concentrations.
• A normal plasma VIP level is 20-30 pmol/L or less, as determined by radioimmunoassay. VIP levels in patients with VIPoma are markedly elevated, often to levels of 160-250 pmol/L or higher. VIP levels are usually 2-10 times the normal range. VIP levels should be drawn after fasting.⁸
  • Because VIP is degraded rapidly, a protease inhibitor, such as aprotinin, is added to the blood sample, which must be kept frozen at -70°C until processed.
  • Because VIP secretion from the tumor may be episodic, collect serum VIP levels during bouts of severe diarrhea. 
• Hypokalemia and non–anion gap acidosis are the main diagnostic features of VIPomas. Hypokalemia may require aggressive replacement.
• Hypercalcemia may occur in the absence of MEN 1 syndrome or elevated parathyroid hormone levels. The mechanism of action is not clear but is believed to be through increased bone resorption. The dehydration from severe diarrhea certainly may exacerbate the hypercalcemia. 
• Hyperglycemia may be caused by the direct glycogenolytic effect of VIP on the liver and by the inhibitory effect of hypokalemia on pancreatic islet cell insulin release.  
• Renal function should be assessed with blood urea nitrogen and serum creatinine levels. 
• Other electrolytes, including magnesium, should be checked and replaced.
• VIPomas may cosecrete other hormones, including pancreatic polypeptide, calcitonin, and neurotensin. 
• Stool weight with potassium measurements verifies high gastrointestinal potassium losses.

Imaging Studies

Imaging studies are focused primarily on the pancreas, where 90% of VIPomas are located. Tumor localization is normally not difficult, because these tumors are generally larger than 3 cm in the longest dimension at the time of diagnosis. The following modalities can be used for imaging VIPomas:

• Computed tomography (CT) scanning
  • CT scanning will successfully identify the primary tumor in most cases.
  • It also assists in including or excluding liver metastasis.
  • In a series of 31 patients from China, Peng and colleagues reported that all VIPomas of the pancreatic body and tail were identified through CT scanning but that only 71% of VIPomas in the pancreatic head were successfully identified with this modality.⁷   
• Magnetic resonance imaging (MRI)
  • MRI may be used if a CT scan is contraindicated, eg, if the patient is allergic to iodine contrast dyes or in cases of renal failure. 
  • VIPomas are observed best on T1-weighted, fat-suppressed images as low – signal-intensity masses.⁹
  • Liver metastases may demonstrate intensive peripheral ring enhancement on immediate post-gadolinium spoiled gradient-echo images.⁹
• Somatostatin receptor scintigraphy
  • Somatostatin receptor scintigraphy may be useful to characterize an abnormality found on a CT scan or to identify occult or distant metastatic disease. It may also be used if postsurgical changes diminish the clarity of a CT scan’s image. 
  • Sensitivity for localization of all pancreatic endocrine tumors has been reported at 80-90%, with 92% sensitivity for tumors larger than 1 cm.
  • Investigations suggest that the use of somatostatin receptor scintigraphy for the localization of neuroendocrine tumors, including VIPomas, may be improved in the future through the employment of single-photon emission CT (SPECT) scanning.¹⁰
• Technetium-99m (^99m Tc) sestamibi - Reports demonstrate successful VIPoma localization with^99m Tc sestamibi.¹¹
• Transabdominal ultrasonography - This may be used for early screening to exclude liver metastases, which may be present as hepatic calcifications.

Other Tests

• Hypochlorhydria or achlorhydria is seen in at least 75% of patients with VIPoma, because vasoactive intestinal polypeptide inhibits the histamine- and pentagastrin-stimulated gastric acid secretion. This can be determined by measuring gastric pH or basal gastric acid output.
• Stool volumes of less than 700 mL virtually exclude the diagnosis. Typical stool volumes are more than 3 L per day.

Procedures

• Endoscopic retrograde cholangiopancreatography
  • This modality may demonstrate occlusion of the major pancreatic duct.
  • Endoscopic retrograde cholangiopancreatography may also demonstrate calcifications in the body of the pancreas.
• Colonoscopy
  
  
  • This may be useful as a means of evaluating for a possible villous adenoma as an alternative cause of potassium-losing diarrhea.

Histologic Findings

VIPomas, like other pancreatic endocrine tumors, are thought to arise from the pluripotent cells in ductal epithelium. Histologic examination usually reveals, as is typical for neuroendocrine tumors, sheets of nested, uniform-appearing cells with round nuclei and a low mitotic rate. Immunohistochemistry staining is positive for chromogranin A and vasoactive intestinal polypeptide. Under electron microscopy, neurosecretory granules may be seen clustering around Golgi complexes and the endoplasmic reticulum. Classifying a tumor as malignant or benign based on microscopic appearance alone is difficult.

Treatment

Medical Care

• Initial treatment for VIPoma is aimed at correcting any volume, electrolyte, and acid-base abnormalities with intravenous normal saline, potassium chloride, and, if acidosis is severe, sodium bicarbonate. In many cases, these abnormalities are severe, requiring hospital admission.
• Somatostatin is highly effective in reducing serum vasoactive intestinal polypeptide levels and promptly controlling diarrhea in more than 90% of patients.⁷ To circumvent the short serum half-life of somatostatin, the derivative octreotide is used. Octreotide is delivered subcutaneously at an initial dose of 50-100 mcg, 3 times a day, which may be titrated up for symptom control to a maximum of 500 mcg, 3 times a day. An available long-acting formulation of octreotide called Sandostatin LAR allows for once monthly intragluteal administration. The starting dose of Sandostatin LAR is 10-20 mg per month. The dose can subsequently be titrated up to a maximum of 40 mg monthly. Diarrhea recurs when Sandostatin treatment is discontinued. It is currently debated whether somatostatin analogues also diminish tumor size.   
• Long-term octreotide treatment frequently results in gradual resistance to this therapy. When maximum tolerable doses of octreotide are unable to control symptoms, interferon alpha may be added to control diarrhea, with a possible modest reduction in tumor size. 
• Glucocorticoids are less effective but less expensive, reducing symptoms in approximately 50% of patients.
• Preoperative treatment with a proton pump inhibitor is advisable to prevent rebound gastric acid hypersecretion after surgical tumor removal. 
• Systemic chemotherapy may be needed in cases of unresectable or progressive disease. Streptozocin, doxorubicin, or fluorouracil, or a combination of these, appears to be beneficial, although the number of treated cases has been limited.
• The use of radiolabeled octreotide to target radiation treatment to VIPoma is based on the affinity of octreotide to the somatostatin receptors on the VIPoma cells. In one trial, half of the patients achieved stabilization of previously progressive tumors, with minimal bone marrow toxicity. This therapeutic approach is still considered experimental.

Surgical Care

• After appropriate fluid and electrolyte replacement, all operable patients with apparently resectable disease should receive abdominal exploration with careful staging. Intraoperative ultrasonography of the pancreas may aid in locating an otherwise unidentified tumor. For patients without nodal or distant metastasis, complete surgical resection offers the only chance for a cure.
  • Local tumor resection is the treatment of choice.^7,12,13,14
  • Pancreatoduodenectomy is indicated when the tumor is in the pancreatic head or processus uncinatus.
• If no tumor is found at surgery, a blind pancreatic tail resection may be performed. A total pancreatectomy no longer is recommended.
• In most cases, metastatic disease is found at the time of diagnosis. For these patients, tumor debulking may reduce clinical symptoms,^7,14 but surgical plans ought to include resection of more than 90% of tumor volume for substantial clinical benefit.
  • Postoperative octreotide therapy will usually be needed indefinitely to control symptoms of vasoactive intestinal polypeptide (VIP) hypersecretion from residual tumor.¹³
  • Unresectable liver metastases may be treated with bland hepatic artery embolization or transcatheter chemoembolization with doxorubicin or cisplatin.¹⁵
  • When embolization is unsuccessful or not feasible for liver metastases, percutaneous or intraoperative radiofrequency tumor ablation may be attempted, although this is not ideal for large metastatic tumors. 
• All patients having surgery should undergo a cholecystectomy to alleviate concerns of gallstones with somatostatin analogue therapy in case such therapy may be needed in the future. 
• Orthotopic liver transplantation has been performed in a small number of select patients with pancreatic endocrine tumors; the 5-year survival rate has been approximately 50%.¹⁶

Consultations

• Consultation with an endocrinologist is indicated, particularly in MEN 1 or other polyhormonal secretion states or if long-term hormonal suppression is required.
• Consultation with a gastroenterologist may be indicated for evaluation of long-term diarrhea or for colonoscopy evaluation for villous adenoma.
• Consultation with a surgeon who is experienced in pancreatic surgery may be indicated if the evaluation suggests a resectable or debulkable tumor or if exploratory surgery is contemplated.

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Somatostatin analogs

These agents may control diarrheal symptoms in as many as 80% of patients with unresectable or metastatic tumors. High-dose treatment may lead to additional, antiproliferative effects. However, the long-term application of somatostatin may down-regulate receptor expression levels, resulting in decreased efficiency despite increasing doses. Short-acting and long-acting depot preparations are available.

Octreotide acetate (Sandostatin)

Acts similarly to the natural hormone somatostatin and has the ability to suppress the secretion of gastroenteropancreatic peptides, including VIP. Start with small doses in patients with VIPomas.

Dosing

Adult

150-300 mcg/24 h SC divided bid/qid (range 150-750 mcg) during initial 2 wk; adjust dose to individual; doses >450 mcg usually not required; LAR long-acting preparation can be used intragluteally once/mo with initial dose of 10-20 mg to maximum of 40 mg monthly; long-acting preparation not for IV/SC administration

Pediatric

Not established

Interactions

Associated with alterations in nutrient absorption; carefully consider effect on any PO drug

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Dosage adjustments may be required to control symptoms; patients may develop hypoglycemia or hyperglycemia due to alterations in balance between counter regulatory hormones; baseline and periodic thyroid function tests advised; gallstones may develop; half-life may be increased in patients with renal failure

Glucocorticoids

Glucocorticoids, which elicit anti-inflammatory properties and cause profound and varied metabolic effects, modify the body's immune response to diverse stimuli. These agents are less effective but are also less expensive; they reduce symptoms in approximately 50% of patients.

Prednisone (Deltasone, Meticorten, Orasone)

Immunosuppressant for the treatment of autoimmune disorders. Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. It stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.

Dosing

Adult

30-50 mg/d PO qd; gradually taper dose following resolution of symptoms

Pediatric

Disease rarely occurs in children

Interactions

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Follow-up

Further Inpatient Care

• Hepatic artery embolization or chemoembolization may be required, as mentioned in Surgical Care.
• Occasionally, external radiation therapy may be indicated in locally unresectable tumors; however, experience with this treatment is limited to a few cases.

Further Outpatient Care

• Patients with VIPomas need frequent outpatient follow-up to monitor hydration status and serum electrolyte levels.
• In patients with continuing fluid loss that is not controlled effectively by medical and surgical treatment options, a Port-a-Cath or other long-term central venous access device may be implanted; the patient may be trained to replace fluid and electrolytes at home or in an ambulatory setting.
• Patients on Sandostatin LAR may need monthly clinic visits to receive the injections.

Inpatient & Outpatient Medications

• Unless a surgical cure has been achieved, octreotide dosing is continued in most patients.
• In patients with advanced disease, tumors may respond to treatment with streptozotocin-based chemotherapy.   
• If conventional chemotherapy and somatostatin are not effective, 5-fluorouracil may be combined with interferon alpha.

Prognosis

• Approximately 50% of surgical patients with VIPoma are cured after tumor resection.
• In a series of 241 patients, the 5-year survival rate was 68.5% for patients with pancreatic VIPomas.¹⁷ The 5-year survival rate for patients without metastatic disease was reported to be about 95%. For patients with metastases, the 5-year survival rate was approximately 60%.

Miscellaneous

Medicolegal Pitfalls

• In patients with VIPoma, parathyroidectomy does not correct hypercalcemia. Vasoactive intestinal polypeptide and its parathyroid hormone – like action cause hypercalcemia.
• Some patients with VIPoma have hypotension due to peripheral vasodilation. Severe hypertension may develop temporarily after tumor removal.

Multimedia

Media file 1: A patient with a large VIPoma. Seen in the image: (A) an arteriogram showing the vascularity of the large VIPoma preoperatively; (B) a large mass seen during surgery; (C) the gross pathologic specimen. The patient subsequently developed liver metastases. He was treated with chemoembolization of the liver masses multiple times and finally succumbed to the disease 20 years after the initial surgical treatment.

References

1. Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. Sep 1958;25(3):374-80. [Medline].

2. Said SI, Mutt V. Polypeptide with broad biological activity: isolation from small intestine. Science. Sep 18 1970;169(951):1217-8. [Medline].

3. Aton SJ, Colwell CS, Harmar AJ, et al. Vasoactive intestinal polypeptide mediates circadian rhythmicity and synchrony in mammalian clock neurons. Nat Neurosci. Apr 2005;8(4):476-83. [Medline]. [Full Text].

4. Doherty GM. Rare endocrine tumours of the GI tract. Best Pract Res Clin Gastroenterol. Oct 2005;19(5):807-17. [Medline].

5. Ayub A, Zafar M, Abdulkareem A, et al. Primary hepatic vipoma. Am J Gastroenterol. Jun 1993;88(6):958-61. [Medline].

6. Grier JF. WDHA (watery diarrhea, hypokalemia, achlorhydria) syndrome: clinical features, diagnosis, and treatment. South Med J. Jan 1995;88(1):22-4. [Medline].

7. Peng SY, Li JT, Liu YB, et al. Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. Pancreas. Jan 2004;28(1):93-7. [Medline].

8. de Herder WW. Biochemistry of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. Mar 2007;21(1):33-41. [Medline].

9. Sofka CM, Semelka RC, Marcos HB, et al. MR imaging of metastatic pancreatic VIPoma. Magn Reson Imaging. 1997;15(10):1205-8. [Medline].

10. Schillaci O, Corleto VD, Annibale B, et al. Single photon emission computed tomography procedure improves accuracy of somatostatin receptor scintigraphy in gastro-entero pancreatic tumours. Ital J Gastroenterol Hepatol. Oct 1999;31 Suppl 2:S186-9. [Medline].

11. Cesani F, Ernst R, Walser E, et al. Tc-99m sestamibi imaging of a pancreatic VIPoma and parathyroid adenoma in a patient with multiple type I endocrine neoplasia. Clin Nucl Med. Jun 1994;19(6):532-4. [Medline].

12. Ruiz-Tovar J, Priego P, Martínez-Molina E, et al. Pancreatic neuroendocrine tumours. Clin Transl Oncol. Aug 2008;10(8):493-7. [Medline].

13. Ghaferi AA, Chojnacki KA, Long WD, et al. Pancreatic VIPomas: subject review and one institutional experience. J Gastrointest Surg. Feb 2008;12(2):382-93. [Medline].

14. Akerstrom G, Hellman P. Surgery on neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. Mar 2007;21(1):87-109. [Medline].

15. King J, Quinn R, Glenn DM, et al. Radioembolization with selective internal radiation microspheres for neuroendocrine liver metastases. Cancer. Sep 1 2008;113(5):921-9. [Medline].

16. Bramley PN, Lodge JP, Losowsky MS, et al. Treatment of metastatic Vipoma by liver transplantation. Clin Transplant. Oct 1990;4(5 part 1):276-8; discussion 279. [Medline].

17. Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res. Dec 1998;17(4):389-400. [Medline].

18. Cellier C, Yaghi C, Cuillerier E, et al. Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. Am J Gastroenterol. Jan 2000;95(1):289-93. [Medline].

19. Jensen RT. Pancreatic endocrine tumors: recent advances. Ann Oncol. 1999;10 Suppl 4:170-6. [Medline].

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22. Remme CA, de Groot GH, Schrijver G. Diagnosis and treatment of VIPoma in a female patient. Eur J Gastroenterol Hepatol. Jan 2006;18(1):93-9. [Medline].

23. Robichon A, Marie JC. Selective photolabeling of high and low affinity binding sites for vasoactive intestinal peptide (VIP): evidence for two classes of covalent VIP-receptor complexes in intestinal cell membranes. Endocrinology. Mar 1987;120(3):978-85. [Medline].

24. Shan L, Nakamura Y, Nakamura M, et al. Somatic mutations of multiple endocrine neoplasia type 1 gene in the sporadic endocrine tumors. Lab Invest. Apr 1998;78(4):471-5. [Medline].

25. Swift PG, Bloom SR, Harris F. Watery diarrhoea and ganglioneuroma with secretion of vasoactive intestinal peptide. Arch Dis Child. Nov 1975;50(11):896-9. [Medline]. [Full Text].

26. Tennvall J, Ljungberg O, Ahren B, et al. Radiotherapy for unresectable endocrine pancreatic carcinomas. Eur J Surg Oncol. Feb 1992;18(1):73-6. [Medline].

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28. Virgolini I, Kurtaran A, Leimer M, et al. Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy. J Nucl Med. Sep 1998;39(9):1575-9. [Medline]. [Full Text].

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Keywords

VIPoma, pancreatic cancer, cancer pancreas, pancreatic cancer symptoms, neuroendocrine tumor, neuroendocrine carcinoma, pancreatic tumor, pancreas tumor, multiple endocrine neoplasia, pancreas tumors, pancreatic tumors, Verner-Morrison syndrome, pancreatic cholera, watery diarrhea-hypokalemia-achlorhydria syndrome, WDHA syndrome, vasoactive intestinal polypeptide, VIP, pancreatic endocrine tumor, multiple endocrine neoplasia type 1 syndrome, MEN 1

Contributor Information and Disclosures

Author

Daniel S Tung, MD, Fellow in Endocrinology, Department of Internal Medicine, Baylor College of Medicine
Daniel S Tung, MD is a member of the following medical societies: American Association of Clinical Endocrinologists and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Sai-Ching Jim Yeung, MD, PhD, FACP, Deputy Section Chief of Emergency Care, Assistant Professor, Department of General Internal Medicine, Ambulatory Treatment and Emergency Care, University of Texas MD Anderson Cancer Center
Sai-Ching Jim Yeung, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Medical Association, American Thyroid Association, and Endocrine Society
Disclosure: Nothing to disclose.

Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine
Klaus Radebold, MD, PhD is a member of the following medical societies: American Gastroenterological Association and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Frederick H Ziel, MD, Associate Professor of Medicine, David Geffen School of Medicine at UCLA; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group
Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS, Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC
Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law Medicine and Ethics, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Related eMedicine topics:
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Neoplasms of the Endocrine Pancreas
Pancreas, Islet Cell Tumors
VIPoma
WDHA Syndrome
Wermer Syndrome (MEN Type 1)

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