VIPomas Clinical Presentation

  • Author: Sai-Ching Jim Yeung, MD, PhD, FACP; Chief Editor: George T Griffing, MD   more...
 
Updated: Jan 4, 2012
 

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, reaching greater than 3 L daily 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.[11] Other studies have also reported facial flushing, but without a reported frequency.

One patient in China reportedly suffered from 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.

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Physical Examination

Physical examination in patients with VIPomas may reveal the following:

  • Tachycardia, decreased skin turgor, and documented weight loss - Resulting from volume depletion
  • Muscle weakness - Caused by marked fecal loss of potassium
  • Mildly distended abdomen
  • Hepatomegaly - May be detected if liver metastasis has occurred
  • Facial flushing - May be seen owing to the the vasodilatory effects of vasoactive intestinal polypeptide (VIP)
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Contributor Information and Disclosures
Author

Sai-Ching Jim Yeung, MD, PhD, FACP  Associate Professor of Medicine, Department of Emergency Medicine, Department of Endocrine Neoplasia and Hormonal Disorders, MD Anderson Cancer Center, University of Texas Medical School at Houston

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.

Coauthor(s)

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.

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.

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.

Additional Contributors

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 Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Frederick H Ziel, MD Associate Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; 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, andInternational Society for Clinical Densitometry

Disclosure: Nothing to disclose.

References
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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.
 
 
 
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