is vipoma a cancer

What is a VIPoma?

A VIPoma, otherwise called a VIPoma, is a tumor made up of non-beta islet cells of the pancreas that secretes vasoactive intestinal peptide (VIP). It is a rare neuroendocrine tumor and affects around 1 in 10,000,000 individuals every year.

What is the difference between a VIPoma and a carcinoid cancer?

Carcinoid occurs when there is an overproduction of serotonin and other hormones. Meanwhile, vipoma is a condition that deals with the production of vasoactive intestinal peptide. In addition, it is a slow growing neuroendocrine cancer that originates in the neuroendocrine cellular system.

What is the prevalence of VIPoma?

A small proportion of tumors secreting VIP have been reported as colorectal cancer, lung cancer, pheochromocytoma, neurofibroma, and ganglioneuroblastoma. Majority of VIPomas occur as isolated tumors, but in about 5% of patients, they are part of the multiple endocrine neoplasia type 1 (MEN1) syndromes.

How is chemotherapy used to treat a VIPoma?

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy tumour cells. You may have chemotherapy if the vipoma has spread to the liver or to other parts of your body.

Are PNETs cancerous?

The group of tumors, formerly known as PNETs, are Grade IV tumors. This means they are malignant (cancerous) and fast-growing.

Is VIPoma treatable?

Surgery. Surgery is the main treatment for VIPomas. There are different types of operations depending on where the tumour is.

How common is VIPoma?

VIPomas are often diagnosed in adults, most commonly around age 50. Women are more likely to be affected than men. This cancer is rare. Each year, only about 1 in 10 million people are diagnosed with a VIPoma.

What causes VIPoma?

The VIPoma syndrome is caused by excessive, unregulated secretion of vasoactive intestinal polypeptide (VIP) by the tumor. However, other substances, such as prostaglandin E2, may occasionally be secreted by the tumors [12].

How do you treat VIPoma?

Initial treatment of VIPomas is directed toward correcting volume and electrolyte abnormalities. Octreotide acetate controls diarrhea in up to 90% of patients with VIPomas. Glucocorticoids reduce symptoms in 50%. Systemic chemotherapy may be needed in cases of unresectable or progressive disease.

What are the symptoms of VIPoma?

Symptoms of VIPoma may include any of the following:Abdominal pain and cramping.Diarrhea (watery, and often in large amounts)Dehydration.Flushing or redness of the face.Muscle cramps due to low blood potassium (hypokalemia)Nausea.Weight loss.

How is VIPoma diagnosed?

Computed tomography (CT), magnetic resonance imaging (MRI), and somatostatin receptor scintigraphy are imaging modalities that can be used in the diagnosis of VIPoma. Reports have demonstrated successful VIPoma localization with99m Tc sestamibi. No formal staging criteria for VIPoma have been generally accepted.

Is VIPoma genetic?

This is often a genetic condition that runs in families. VIPoma: A type of neuroendocrine pancreatic tumor that secretes vasoactive intestinal polypeptide (VIP). This leads to severe intermittent diarrhea that causes further problems, including dramatic potassium loss.

Can you have a VIPoma without diarrhea?

Vasoactive intestinal polypeptide-secreting tumors (VIPoma) are a rare pancreatic neuroendocrine tumor that can cause chronic diarrhea with 1 case per 10 million people per year.

What causes high VIP levels?

A very high level is usually caused by a VIPoma. This is an extremely rare tumor that releases VIP. VIP is a substance found in cells throughout the body. The highest levels are normally found in cells in the nervous system and gut.

Which is known as VIP disease?

The VIPoma syndrome, also called the Verner-Morrison syndrome, pancreatic cholera, and the WDHA syndrome (for watery diarrhea, hypokalemia, and achlorhydria), results from an endocrine tumor, usually in the pancreas that ectopically secretes vasoactive intestinal polypeptide (VIP).

What does VIP hormone do?

A hormone found in the pancreas, intestine, and central nervous system. It has many actions in the body, such as helping to control the secretion of water, salts, enzymes, and gastric acid during digestion. It also causes smooth muscles in the digestive tract, the heart, and the blood vessels to relax.

Can you have a VIPoma without diarrhea?

Vasoactive intestinal polypeptide-secreting tumors (VIPoma) are a rare pancreatic neuroendocrine tumor that can cause chronic diarrhea with 1 case per 10 million people per year.

How do you diagnose VIPoma?

A diagnosis of VIPoma is made when watery diarrhea, hypokalemia, and achlorhydria are present in the setting of elevated serum VIP concentrations. Stool volumes of less than 700 mL/day virtually exclude the diagnosis; typical stool volumes in the presence of a VIPoma exceed 3 L/day.

Is VIPoma genetic?

This is often a genetic condition that runs in families. VIPoma: A type of neuroendocrine pancreatic tumor that secretes vasoactive intestinal polypeptide (VIP). This leads to severe intermittent diarrhea that causes further problems, including dramatic potassium loss.

What causes high VIP levels?

A very high level is usually caused by a VIPoma. This is an extremely rare tumor that releases VIP. VIP is a substance found in cells throughout the body. The highest levels are normally found in cells in the nervous system and gut.

What is the cause of vipoma?

The exact cause of VIPomas is not known. VIPoma causes cells in the pancreas to produce a high level of a hormone called vasoactive intestinal peptide (VIP). VIP is a neurohormone produced in the central nervous system as well as in the neurons of the gastrointestinal (GI), respiratory, and urogenital tracts. Vasoactive intestinal peptide (VIP) hormone increases secretions from the intestines. Vasoactive intestinal peptide (VIP) functions as a vasodilator and regulator of smooth muscle activity in the gastrointestinal system, stimulator of water and electrolyte secretion from the intestinal tract, an inhibitor of gastric acid secretion, and promotor of blood flow mainly in the gastrointestinal (GI) tract.

How long do people with vipoma live?

This is called overall survival. This study found that people with VIPoma live about 8 years on average after being diagnosed. The median survival of patients with VIPoma is 96 months 20).

What is the name of the tumor that secretes vasoactive peptides?

Vipoma. VIPoma is short for vasoactive intestinal peptide tumor also called Verner Morrison syndrome, is a rare type of pancreatic neuroendocrine cancer that secretes vasoactive intestinal peptide (VIP) – a hormone that stimulates the secretion and inhibits the absorption of sodium, chloride, potassium and water within the small intestine, ...

What is the name of the disorder that is caused by high levels of the hormone VIP?

The VIPoma syndrome is also known as Verner-Morrison syndrome or WDHA syndrome (watery diarrhea, hypokalemia, and hypochlorhydria or achlorhydria syndrome) and pancreatic cholera syndrome 7). Verner Morrison syndrome is the name given to the collection of symptoms caused by having high levels of the hormone VIP (vasoactive intestinal peptide).

What is the name of the pancreatic tumor that causes watery diarrhea?

The VIPoma syndrome is also known as Verner-Morrison syndrome or WDHA syndrome (watery diarrhea, hypokalemia, ...

How does a vipoma work?

For a VIPoma in your lung, your surgeon removes the tumour and checks for other tumours. You might just have the tumour removed. Or you may need to have part of the lung removed.

What drugs can you take for a VIPoma?

You may have 2 types of targeted drugs called everolimus and sunitinib if your VIPoma has started in the pancreas.

What is a vipoma?

A VIPoma or vipoma ( / vɪˈpoʊmə /) is a rare endocrine tumor that overproduces vasoactive intestinal peptide (thus VIP + -oma ). The incidence is about 1 per 10,000,000 per year. VIPomas usually (about 90%) originate from the non-β islet cells of the pancreas. They are sometimes associated with multiple endocrine neoplasia type 1. Roughly 50–75% of VIPomas are malignant, but even when they are benign, they are problematic because they tend to cause a specific syndrome: the massive amounts of VIP cause a syndrome of profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria, acidosis, flushing and hypotension (from vasodilation ), hypercalcemia, and hyperglycemia. This syndrome is called Verner–Morrison syndrome ( VMS ), WDHA syndrome (from watery diarrhea–hypokalemia–achlorhydria), or pancreatic cholera syndrome ( PCS ). The eponym reflects the physicians who first described the syndrome.

Can a vipoma be cured?

Surgery can usually cure VIPomas. However, in one-third to one-half of patients, the tumor has spread by the time of diagnosis and cannot be cured.

Can a fasting plasma level confirm a tumor?

Besides the clinical picture, fasting VIP plasma level may confirm the diagnosis, and CT scan and somatostatin receptor scintigraphy are used to localise the tumor, which is usually metastatic at presentation.

What is a vipoma?

VIPomas are rare cancers that start in the neuroendocrine cells that make the hormone VIP. VIP stands for vasoactive intestinal peptide. It relaxes the muscles in the stomach and bowel, and helps to control the balance of sugar, salt and water in the gastrointestinal (GI) tract.

How big is a vipoma?

It is about 6 inches (15 centimeters) and shaped like a leaf. The pancreas has 3 parts: The pancreatic duct is a tube that collects the digestive juices made by the pancreas and carries them into the first part of the bowel (duodenum). Most VIPomas start in the tail of the pancreas.

Is a vipoma a cancer?

All VIPomas are cancers. VIPomas often grow slowly and are diagnosed early. But some people are diagnosed when their cancer has already spread to other parts of the body (secondary tumours or metastases).

What are VIPomas?

VIPomas are rare NETs that start in neuroendocrine cells that make the hormone VIP.

Where do vipomas start?

VIPomas are neuroendocrine tumours (NETs) that usually start in the pancreas. They can also start in other parts of the body such as the bowel, lungs and liver. They are a type of functioning NETs of the pancreas.

What tests are needed to diagnose vipoma?

You have some tests to diagnose a VIPoma. This might include blood tests and scans.

How to treat vipoma?

The first step in the treatment of a patient with a VIPoma is to correct the fluid and electrolyte deficits. In severe cases, intravenous resuscitation with potassium and bicarbonate can be required. Administration of a somatostatin analog (SSA) can decrease the secretory diarrhea, further aiding in the restoration of fluid and electrolyte imbalances (22). In the acute setting, octreotide can be administered subcutaneously, or via continuous intravenous infusion.

Where are VIPomas located?

VIPomas are usually located in the pancreas (75%), or along the sympathetic chain as seen in ganglioneuromas, ganglioneuroblastomas, or neuroblastomas. Pheochromocytomas secreting VIP have also been described (18). Extrapancreatic neurogenic VIPomas are most often diagnosed in children. As the primary pancreatic tumor is larger than 2cm in 80% of VIPomas (19), they can be easily detected with MRI, CT, or endoscopic ultrasound (EUS). Additionally, a positron emission tomography (PET)-CT with gallium-labelled somatostatin analogs (DOTATATE, DOTANOC, DOTATOC) should be performed. In a small series with 11 patients, somatostatin receptor scintigraphy was superior to conventional radiology in detecting a VIPoma (20). Similar to work-up for all NENs, a biopsy is advised to confirm the diagnosis and for grading (Ki67 index), as the grade can influence treatment decisions (21). An overview of the current pancreatic NEN (panNEN) staging and grading systems is provided in the chapter “Insulinoma”.

What is a vip?

Vasoactive intestinal peptide (VIP) is a neurotransmitter which is present in the neurons in the central nervous system, the lung, intestine, adrenals, pancreas, and liver and in neuroendocrine cells in the pancreas. In the gastrointestinal tract, VIP stimulates contraction of enteric smooth muscle cells, secretion from the exocrine pancreas, gastrointestinal blood flow, and inhibition of gastric acid secretion. A VIPoma is a neuroendocrine neoplasm secreting VIP, causing severe watery diarrhea, which can result in hypokalemia and metabolic acidosis. Larger tumors (with highly elevated plasma VIP levels) can cause up to 6-8L of watery stools per day. Other symptoms include hypochlorhydria, stimulation of glycogenolysis, facial flushing, and hypercalcemia. By definition, plasma VIP levels are elevated in all patients with the VIPoma syndrome. VIPomas are usually located in the pancreas (75%) or along the sympathetic chain as seen in ganglioneuromas, ganglioneuroblastomas, or neuroblastomas. The first step in the treatment of a patient with a VIPoma is to correct the fluid and electrolyte deficits. Administration of a somatostatin analog (SSA) can decrease diarrhea, further aiding in the restoration of fluid and electrolyte imbalances. In patients with a metastatic or unresectable VIPoma, SSAs likely prolong progression-free survival. Other treatment options include peptide receptor radionuclide therapy (PRRT) with radiolabeled SSAs (177Lu-DOTATATE), everolimus, sunitinib, cytotoxic chemotherapy, or liver-directed therapies. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.

What is the half life of a vip?

In the circulation, VIP has a half-life of less than 1 minute and normally plasma levels of VIP are low (<20pmol/L) (13,14). In the absence of a VIPoma, VIP in plasma originates from VIP-containing nerve fibers and reflects the overflow of VIP from vascular nerves. By definition, plasma VIP levels are elevated in all patients with the VIPoma syndrome. Bloom and colleagues measured plasma VIP levels in nearly 1000 patients with diarrhea and all patients with plasma VIP levels greater than 60pmol/L indeed had a VIPoma (13). Moderately elevated plasma VIP levels can incidentally also be caused by gastrointestinal ischemia, renal insufficiency, or congestive heart failure (15-17).

Where is the vip found?

Vasoactive intestinal polypeptide (VIP) is a neurotransmitter found in the central nervous system, in neurons in the intestine, lungs, adrenals, pancreas and liver and in neuroendocrine cells in the pancreas (1). VIP has been localized in axons, dendrites, neuronal cell bodies and presynaptic nerve terminals from where VIP is released (1). In the gastrointestinal tract, VIP stimulates contraction of enteric smooth muscle cells, secretion from the exocrine pancreas, gastrointestinal blood flow, and inhibition of gastric acid secretion (2-4). A VIPoma is a neuroendocrine neoplasm (NEN) secreting VIP, causing severe watery secretory diarrhea, which can result in hypokalemia and metabolic acidosis.

What are the symptoms of a vipoma?

This causes secretory diarrhea which is the most characteristic symptom of a VIPoma. In severe cases patients can produce over 6-8L of stools per day. The stool is rich in electrolytes like potassium and bicarbonate, resulting in hypokalemia and metabolic acidosis. Other symptoms include hypochlorhydria, stimulation of glycogenolysis, facial flushing, and hypercalcemia. VIP has a structural homology with secretin, glucagon, and GIP which may account for enhanced secretion of pancreatic enzymes, inhibition of gastric acid secretion, and glycogenolysis (9). Nearly 15-30% of patients present with facial flushing (10). The cause of this patchy erythematous flushing is not clear, but it has been attributed to VIP, or prostaglandins, which may be co-secreted by the tumor. Around 50% of patients have hypercalcemia, but again the mechanism is unknown. The incidence of VIPomas is estimated to be 1–2 per 10 million per year (11). VIPomas can be associated with multiple endocrine neoplasia 1 (MEN1), but they remain rare presenting in less than 1% of patients with MEN1 (12).

What is the treatment for unresectable vipoma?

In case of an unresectable VIPoma, treatment is focused on tumor stabilization and control of VIP secretion. In general, anti-tumor therapy is similar to non-functioning panNENs as described in the guidelines by ENETS, NANETS and ESMO (24-26).

What is a vipoma?

Vipoma (Verner-Morrison syndrome, pancreatic cholera, WDHA syndrome – for watery diarrhea, hypokalemia, and achlorhydria) is one of several rare types of functioning pancreatic neuroendocrine tumors (NETs) and accounts for 2-4% of all pancreatic NETs. Most cases are sporadic, but about 10% are associated with multiple endocrine neoplasia type 1 (MEN1). Vipomas are usually solitary tumors, larger than 3 cm in diameter, and occur in an intrapancreatic location in more than 95% of adult cases (tail of pancreas in 75% of cases). In children, vipomas occur in sympathetic ganglia and in the adrenal glands. Metastatic disease at the time of diagnosis is present in 70-80% of cases, but, as in other NETs, the degree of malignancy cannot be predicted by histological appearance.

What is the symptom of vasoactive intestinal polypeptide?

Symptoms associated with dehydration and hypokalemia include lethargy, nausea, vomiting, muscle weakness, and muscle cramps. Abdominal pain is mild or absent. Flushing episodes can occur (20% of cases) and are attributable to the vasodilator action of VIP. Other common findings include hyperglycemia and hypercalcemia.

Can you test for vipoma with a single elevated fasting concentration?

A single elevated fasting VIP concentration in the presence of secretory diarrhea should be repeated to establish the diagnosis of vipoma.

What Is Vipoma?

• Vipomais an acronym which stands for vasoactive intestinal polypeptide (VIP), which is otherwise known as Verner Morrison syndrome. Other experts would call it pancreatic cholera. One clinical feature is heavy diarrhea. It is actually a rare tumor of the endocrine system which originates in the pancreas, where vasoactive intestinal peptide is produ...

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Vipoma Versus Carcinoid