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ViPoma

Updated : February 21, 2024





Background

Vasoactive intestinal peptide (VIP) tumors, often known as VIPomas, are neuroendocrine tumors that secrete VIP in an uncontrolled way. They were first identified as a pancreatic tumors causing hypokalemia and watery diarrhea by Werner and Morrison in 1958. The trio of Bloom, Pearse, and Polak established the mediator’s identity in 1973.

Other names for VIPoma syndrome include Verner-Morrison syndrome, pancreatic cholera syndrome, watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome or hypochlorhydria. The clinical manifestations, diagnostics, and medication are highlighted in this study.

Epidemiology

VIPomas are uncommon tumors that can affect both adults and children. Their incidence ranges from 0.05 percent to 2.0 percent. They most frequently affect adults over the age of 30 and 50, and 95 percent of cases are intrapancreatic. A small proportion of tumors that secrete VIP has already been linked to pheochromocytoma, lung disease, neurofibroma, and ganglioneuroblastomas.

While about 5 percent of patients have VIPomas as a component of the MEN1 (multiple endocrine neoplasia type 1) syndromes, VIPomas typically develop as separate tumors. When diagnosed, more than 50 percent of VIPomas already had metastasized.

They are commonly diagnosed in youngsters between the years of 2 and 4. Most of the VIPomas in kids are either ganglioneuroblastoma or ganglioneuroma, which develop from the sympathetic ganglia’s neural crest tissues in the retroperitoneum or mediastinum. They could come from the adrenal medulla as well.

Anatomy

Pathophysiology

The Gastrointestinal system is the major main organ that is impacted by excessive VIP production out from tumors, among other major organs. VIP contains 28 amino acids and is a neurotransmitter from the secretin-glucagon class.

It is a powerful inhibitor of gastric acid secretion and a stimulant of intestinal cAMP (cyclic adenosine monophosphate) synthesis. Bone resorption, lipolysis, glycogenolysis, and vasodilation are all encouraged.

The Gastrointestinal epithelial cells secrete a lot of water and electrolytes, which has downstream effects such as hypercalcemia, hypokalemia, decreased acid secretion, raised blood sugar, and face flushing.

Etiology

The CNS, as well as the neurons in the GI, urogenital tracts, and respiratory, all produce VIP, a neurohormone.

Its activities include vasodilation, smooth muscle activation, stimulation of intestinal water and electrolytes secretion, inhibition of gastric acid production, and promotion of blood flow, primarily in the Gastrointestinal system.

Genetics

Prognostic Factors

The average patient survival time for VIPoma is ninety-six months. The degree, tumor stage, and ability to be surgically removed from the tumor all play a major role.

According to the most updated National Comprehensive Cancer Network (NCCN) regulations, the first three to twelve-month post-resection follow-up comprises a history and physical examination, multiphasic CT scan and MRI, and blood VIP concentration.

It is advised to continue with the same steps every six to twelve months after the first year.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK507698/

ViPoma

Updated : February 21, 2024




Vasoactive intestinal peptide (VIP) tumors, often known as VIPomas, are neuroendocrine tumors that secrete VIP in an uncontrolled way. They were first identified as a pancreatic tumors causing hypokalemia and watery diarrhea by Werner and Morrison in 1958. The trio of Bloom, Pearse, and Polak established the mediator’s identity in 1973.

Other names for VIPoma syndrome include Verner-Morrison syndrome, pancreatic cholera syndrome, watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome or hypochlorhydria. The clinical manifestations, diagnostics, and medication are highlighted in this study.

VIPomas are uncommon tumors that can affect both adults and children. Their incidence ranges from 0.05 percent to 2.0 percent. They most frequently affect adults over the age of 30 and 50, and 95 percent of cases are intrapancreatic. A small proportion of tumors that secrete VIP has already been linked to pheochromocytoma, lung disease, neurofibroma, and ganglioneuroblastomas.

While about 5 percent of patients have VIPomas as a component of the MEN1 (multiple endocrine neoplasia type 1) syndromes, VIPomas typically develop as separate tumors. When diagnosed, more than 50 percent of VIPomas already had metastasized.

They are commonly diagnosed in youngsters between the years of 2 and 4. Most of the VIPomas in kids are either ganglioneuroblastoma or ganglioneuroma, which develop from the sympathetic ganglia’s neural crest tissues in the retroperitoneum or mediastinum. They could come from the adrenal medulla as well.

The Gastrointestinal system is the major main organ that is impacted by excessive VIP production out from tumors, among other major organs. VIP contains 28 amino acids and is a neurotransmitter from the secretin-glucagon class.

It is a powerful inhibitor of gastric acid secretion and a stimulant of intestinal cAMP (cyclic adenosine monophosphate) synthesis. Bone resorption, lipolysis, glycogenolysis, and vasodilation are all encouraged.

The Gastrointestinal epithelial cells secrete a lot of water and electrolytes, which has downstream effects such as hypercalcemia, hypokalemia, decreased acid secretion, raised blood sugar, and face flushing.

The CNS, as well as the neurons in the GI, urogenital tracts, and respiratory, all produce VIP, a neurohormone.

Its activities include vasodilation, smooth muscle activation, stimulation of intestinal water and electrolytes secretion, inhibition of gastric acid production, and promotion of blood flow, primarily in the Gastrointestinal system.

The average patient survival time for VIPoma is ninety-six months. The degree, tumor stage, and ability to be surgically removed from the tumor all play a major role.

According to the most updated National Comprehensive Cancer Network (NCCN) regulations, the first three to twelve-month post-resection follow-up comprises a history and physical examination, multiphasic CT scan and MRI, and blood VIP concentration.

It is advised to continue with the same steps every six to twelve months after the first year.

https://www.ncbi.nlm.nih.gov/books/NBK507698/