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VIPoma of Pancreas

Last updated Jan. 8, 2019

Approved by: Maulik P. Purohit MD MPH

VIPoma of Pancreas is an extremely rare endocrine tumor of pancreas that secretes vasoactive intestinal peptide (VIP, a hormone).


What are the other Names for this Condition? (Also known as/Synonyms)

  • Pancreatic VIPoma
  • Vasoactive Intestinal Peptide Secreting Tumor of Pancreas
  • Verner-Morrison Syndrome

What is VIPoma of Pancreas? (Definition/Background Information)

  • VIPoma of Pancreas is an extremely rare endocrine tumor of pancreas that secretes vasoactive intestinal peptide (VIP, a hormone). Normally, VIP is secreted by nerves of the central nervous system and nerves in the GI tract, respiratory tract, and urogenital tract
  • 90% of VIPomas arise in the pancreas, while the remaining 10% occur in the gastrointestinal (GI) tract. In the GI tract, the colon is the frequent site of this malignant tumor. In some very rare cases, the tumor is present in the adrenal gland or liver (there is a 1-2% chance of the tumor arising from these locations)
  • VIPoma of Pancreas generally occurs in middle-aged and slightly older individuals, though it may affect children too. These tumors may be small and during the early stages may not cause any significant signs and symptoms
  • VIPoma of Pancreas can cause severe watery diarrhea that is longstanding, severe loss of body fluids, and increased blood calcium and glucose levels. Most tumors may have metastasized during diagnosis to the liver or lungs
  • In majority of cases, a complete surgical excision is the preferred mode of treating this malignant tumor. In cases of metastasis (which takes place frequently), a combination of chemotherapy, radiation therapy, and surgical procedures may be used
  • The prognosis of Pancreatic VIPoma depends upon many factors including the stage of the tumor, the age and overall health of the individual; however, the prognosis is unpredictable

The pancreas is an important organ of the digestive system.

  • Based on function of the pancreas, it can be functionally divided into 2 parts, namely:
    • Exocrine pancreas, which produces digestive enzymes, and
    • Endocrine pancreas that produces hormones such as insulin, glucagon, and somatostatin. Insulin and glucagon helps with controlling sugar levels in blood
  • 95% of the pancreas is exocrine portion and 5% is endocrine portion. Pancreatic tumors (both benign and malignant) can arise from both the exocrine and the endocrine part
  • Based upon anatomy of the pancreas, it can be divided into 3 main parts, namely the:
    • Head,
    • Body, and
    • Tail
  • Pancreatic tumors can affect the head, body, and tail region of the pancreas. Some tumors can affect one area of the pancreas more than the other areas.

Hence, localizing the tumor site can guide the healthcare provider to arrive at a probable diagnosis.

Who gets VIPoma of Pancreas? (Age and Sex Distribution)

  • VIPoma of Pancreas is an extremely rare and malignant tumor that generally occurs in adults in the age range of 40-50 years (5th decade). However, a much wider age range is observed and even children may be affected
  • The incidence of the tumor is 1 in 10 million among the general population
  • It can affect both males and females; though, in children the male-female ratio is 1:1, while in adults the male-female ratio is 1:3
  • The condition can occur worldwide; individuals of all racial and ethnic background may be affected

What are the Risk Factors for VIPoma of Pancreas? (Predisposing Factors)

The specific risk factors for VIPoma of Pancreas are generally unknown or unidentified. However, some scientists believe that the following factors may increase the risk.

  • Multiple endocrine neoplasia type 1 (MEN 1, an inherited genetic disorder): About 5% of the cases are seen in individuals with this syndrome, and the tumor arises from non beta cells of the pancreas
  • A family history of VIPoma or other pancreatic tumors

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of VIPoma of Pancreas? (Etiology)

  • The exact cause of VIPoma of Pancreas development is unknown
  • Research scientists believe that the cause of the condition is mostly due to molecular or genetic mutations, which results in tumor formation

What are the Signs and Symptoms of VIPoma of Pancreas?

The signs and symptoms of VIPoma of Pancreas depend upon the size and location of the tumor. During the initial stages, small tumors may not cause any signs and symptoms that are readily recognized. Hence, these tumors are only detected incidentally, when being worked-up for other conditions (i.e., diagnostic tests and exams undertaken for other health conditions).

The signs and symptoms may include the following:

  • Severe chronic watery diarrhea that could last for several years (some individuals may have had diarrhea for up to 5 years, prior to diagnosis)
  • Severe dehydration
  • Flushing due to vasodilatation
  • Decreased blood pressure (hypotension)
  • Increased serum calcium (hypercalcemia)
  • Increased blood glucose (hyperglycemia)
  • Muscle weakness
  • Crampy abdominal pain, back pain
  • Signs and symptoms of any underlying disorder, such as multiple endocrine neoplasia type 1

General features of VIPoma of Pancreas include:

  • This malignant tumor is present in the body and tail, more than in the head of the pancreas. They can arise from the endocrine portion of the pancreas
  • Mostly (in 95% of the cases), the tumors are solitary
  • The average tumor size in the pancreas is 3 cm (they are usually small in size)

How is VIPoma of Pancreas Diagnosed?VIPoma of Pancreas Diagnosed?

Frequently, pancreatic tumors are difficult to detect/diagnose in the early stages. The signs and symptoms can be very similar to other conditions. Since the pancreas is located in the peritoneum, behind many organs, there is a lot of space for the tumor to grow (often unnoticed).

The following are the diagnostic methods that may be used to detect VIPoma of Pancreas:

  • A thorough physical examination and a complete medical history is very important
  • Comprehensive metabolic panel of blood
  • Blood test to check for serum amylase levels
  • Complete blood count with differential
  • Fasting VIP plasma levels
  • Vasoactive intestinal peptide (VIP) level testing using selective transhepatic portal venous sampling. In this test, the VIP levels from various parts of the portal system is measured to localize the tumor
  • Blood tests that may involve tumors markers, such as:
    • Carcinoembryonic antigen (CEA)
    • CA 19.9
    • CA 15.3
    • Alpha fetoprotein
  • Radiological studies that may include:
    • Abdominal ultrasound: A procedure where high-frequency sound waves are used to produce real-time images
    • Endoscopic ultrasound: It is a minimally-invasive procedure that uses high-frequency sound waves to obtain detailed images of the pancreas
    • CT scan of abdomen: It may be helpful in detecting recurrences, or if metastasis to other organs has occurred
    • MRI scan of abdomen: It helps produce high-quality pictures of certain body parts including the tissues, muscles, nerves, and bones
  • Additionally, the following magnetic resonance imaging procedures may be used particularly in individuals suspected of having pancreatic tumor/cancer:
    • MR cholangio-pancreatography (MRCP)
    • MR angiography (MRA)
  • Positron emission tomography (PET) scan: It is particularly helpful in visualizing the spread of the cancer to other body parts (metastasis) and/or recurrence
  • Percutaneous transhepatic cholangiography (PTC): A contrast material is injected into the bile ducts to visualize the structure
  • Endoscopic retrograde cholangio-pancreatography (ERCP): This technique is used when symptoms of pancreatic cancer are present in the individual. A special equipment and dye injections are used to obtain a series of images. If required, a biopsy sample may also be collected during the procedure
  • Invasive diagnostic procedures such as:
    • Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
    • Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’
  • Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination to the pathologist, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

The tissue for diagnosis can be procured in multiple different ways, and they include:

  • Fine needle aspiration (FNA) biopsy of the pancreatic tumor:
    • A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of the pancreatic tumor
  • Open biopsy of the pancreatic tumor

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of VIPoma of Pancreas?

The complications due to VIPoma of Pancreas may include the following:

  • Gastrointestinal obstruction
  • Biliary tract obstruction causing jaundice
  • Compress adjoining organs if the tumor size is large, which may cause the organs to dysfunction or function abnormally
  • Chronic renal failure
  • Metabolic imbalance caused by loss of electrolytes
  • In children, failure to thrive
  • The tumor is usually metastatic at the time of diagnosis (in 60-80% of the cases). Metastasis can occur to the liver, lymph nodes, lung, and kidney
  • The tumor can recur after treatment
  • Complications may arise due to the presence of the underlying genetic disorder (MEN type 1)

How is VIPoma of Pancreas Treated?

The treatment measures available for VIPoma of Pancreas include the following:

  • Stabilize the patient by treating severe dehydration
  • Octreotide (medication) can block the effect of VIPoma
  • A majority of patients can be cured through surgery, if the tumor is confined to the pancreas
  • In over 2/3rds of the individuals (in whom metastasis is noted), the treatment is aimed at controlling the symptoms, since the condition cannot be cured
  • If the tumor has metastasized, then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor. This is often based upon the individual’s specific circumstance
  • Peptide receptor radionuclide therapy (PRRT) is a new method to treat VIPoma (which has undergone metastasis)
  • Other treatment options may include:
    • Chemotherapy-radiotherapy: Where a combination therapy is used to treat the cancer
    • Targeted therapy: It used to target abnormal cancer cells and reduce the chance of cancer spreading to other parts of the body
    • Biological therapy: Also, known as immunotherapy, where certain bacteria or vaccines are used to indirectly treat the cancer
  • Treatment of the underlying MEN type 1 (if present)
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Long-term monitoring is important following treatment
  • Monitoring of serum VIP levels can help detect early recurrence

How can VIPoma of Pancreas be Prevented?

Currently, there are no specific methods or guidelines to prevent VIPoma of Pancreas. However, the condition may be avoided through the following measures:

  • Genetic testing in individuals with a family history of pancreatic tumors or other underlying genetic conditions associated with pancreatic tumors
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations for those who have already endured the tumor

What is the Prognosis of VIPoma of Pancreas? (Outcomes/Resolutions)

  • VIPoma of Pancreas is a malignant tumor and its prognosis depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) 
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared to those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have a poorer prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond so well to treatment
  • An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
  • In general, the prognosis of VIPoma of Pancreas is unpredictable. Even though in majority of cases the prognosis is good, in case of metastasis, the condition is often difficult to cure
  • In case of underlying genetic syndromes, such as multiple endocrine neoplasia 1, the prognosis is further dependent upon the severity of the syndrome
  • Regular follow up visits with the healthcare provider are important; long-term observation and monitor of the individual is necessary

Additional and Relevant Useful Information for VIPoma of Pancreas:

It is estimated that the average lifetime risk of developing pancreatic cancer is about 1.5%.

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Nov. 27, 2015
Last updated: Jan. 8, 2019