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{{Short description|A tumor that often causes a syndrome of diarrhea and electrolyte imbalance}}
{{Short description|A rare neuroendocrine tumor that causes watery diarrhea and electrolyte imbalance}}
{{Infobox medical condition (new)
{{Infobox medical condition
| name           = VIPoma  
| name = VIPoma
| synonyms       =
| synonyms = Verner–Morrison syndrome, Pancreatic cholera, WDHA syndrome (Watery Diarrhea, Hypokalemia, and Achlorhydria)
| image          =
| pronounce = {{IPAc-en|v|ɪ|ˈ|p|oʊ|m|ə}}
| caption        = 
| field = [[Oncology]], [[Endocrinology]]
| pronounce      = 
| symptoms = Profuse watery diarrhea, dehydration, flushing, cramping, weakness
| field           = [[Oncology]]  
| complications = [[Hypokalemia]], [[achlorhydria]], [[metabolic acidosis]], [[renal failure]], [[malnutrition]]
|
| onset = Typically adults aged 30–50
| symptoms       =  
| duration = Chronic unless treated
| complications   =  
| types = Pancreatic or extrapancreatic neuroendocrine tumor
| onset           =  
| causes = [[Neoplasm]] of VIP-secreting cells
| duration       =  
| risks = [[Multiple endocrine neoplasia type 1]]
| types           =  
| diagnosis = [[Plasma]] VIP level, imaging ([[CT scan]], [[MRI]], [[somatostatin receptor scintigraphy]])
| causes         =  
| differential = [[Cholera]], [[Carcinoid syndrome]], [[laxative abuse]], [[Zollinger–Ellison syndrome]]
| risks           =  
| prevention = None known
| diagnosis       =  
| treatment = [[Surgical resection]], [[somatostatin analogs]], supportive care
| differential   =  
| medication = [[Octreotide]], [[Lanreotide]], electrolyte replacement
| prevention     =  
| prognosis = Variable; often poor in metastatic cases
| treatment       =  
| frequency = ~1 per 10 million annually
| medication     =  
| deaths = Associated with complications of metastatic disease or severe dehydration
| prognosis       =  
| frequency       =  
| deaths         =  
}}
}}
'''VIPoma''' is a rare type of [[neuroendocrine tumor]] that secretes excessive amounts of [[vasoactive intestinal peptide]] (VIP), a hormone involved in regulating intestinal water and electrolyte secretion. The excessive VIP leads to a distinct clinical syndrome characterized by profound watery diarrhea, hypokalemia (low potassium), and achlorhydria (lack of stomach acid), collectively known as '''WDHA syndrome''' or '''Verner–Morrison syndrome'''. It is also colloquially referred to as '''pancreatic cholera''' due to the similarity of symptoms to [[cholera]] infection.


A '''VIPoma''' or '''vipoma''' ({{IPAc-en|v|ɪ|ˈ|p|oʊ|m|ə}}) is a rare [[endocrine oncology|endocrine tumor]]<ref>{{DorlandsDict|nine/000116307|VIPoma}}</ref> that overproduces [[vasoactive intestinal peptide]] (thus ''VIP'' + ''[[wikt:-oma#Suffix|-oma]]''). The [[Incidence (epidemiology)|incidence]] is about 1 per 10,000,000 per year. VIPomas usually (about 90%) originate from the [[pancreatic islets|non-β islet cells]] of the [[pancreas]]. They are sometimes associated with [[multiple endocrine neoplasia type 1]]. Roughly 50%-75% of VIPomas are [[cancer|malignant]], but even when they are [[benign tumor|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 (physiology)|flushing]] and [[hypotension]] (from [[vasodilation]]), [[hypercalcemia]], and [[hyperglycemia]].<ref>{{cite journal |vauthors=Mansour JC, Chen H |title=Pancreatic endocrine tumors |journal=J Surg Res |volume=120 |issue=1 |pages=139–61 |date=Jul 2004 |pmid=15172200 |doi=10.1016/j.jss.2003.12.007 |url=}}</ref><ref>{{Cite web|url=https://rarediseases.info.nih.gov/diseases/5493/vipoma|title=VIPoma {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program|website=rarediseases.info.nih.gov|language=en|access-date=2018-04-17}}</ref> This syndrome is called '''Verner–Morrison syndrome''' ('''VMS'''), '''WDHA syndrome''' (from watery diarrhea–hypokalemia–achlorhydria), or '''pancreatic cholera syndrome''' ('''PCS'''). The [[medical eponyms|eponym]] reflects the physicians who first described the syndrome.<ref>{{cite journal |vauthors=Verner JV, Morrison AB |title=Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia |journal=Am J Med |volume=25 |issue=3 |pages=374–80 |date=Sep 1958 |pmid=13571250 |doi= 10.1016/0002-9343(58)90075-5}}</ref>
== Pathophysiology ==
VIPomas most commonly originate from the [[pancreas]], particularly the [[non-β islet cells]], although rare cases have been found in extrapancreatic sites such as the [[bronchus]], [[adrenal gland]], or [[colon]]. Approximately 50–75% of VIPomas are [[malignant tumor|malignant]] at the time of diagnosis, frequently with metastasis to the [[liver]] or [[lymph nodes]].


==Symptoms and signs==
The excessive VIP causes increased intestinal secretion of water and electrolytes, reduced gastric acid secretion, and smooth muscle relaxation, resulting in:
The major clinical features are prolonged watery [[diarrhea]] (fasting stool volume > 750 to 1000 mL/day) and symptoms of [[hypokalemia]] and [[dehydration]].
Half of the patients have relatively constant [[diarrhea]] while the rest have alternating periods of severe and moderate [[diarrhea]].
One third have diarrhea < 1yr before diagnosis, but in 25%, diarrhea is present for 5 yr or more before diagnosis.
[[Lethargy]], muscle weakness, [[nausea]], vomiting and crampy abdominal pain are frequent symptoms.
[[Hypokalemia]] and impaired [[glucose]] tolerance occur in < 50% of patients. [[Achlorhydria]] is also a feature.
During attacks of diarrhea, flushing similar to the [[carcinoid syndrome]] occur rarely.{{cn|date=March 2019}}


==Diagnosis==
* Chronic watery diarrhea (often exceeding 3 liters/day)
Besides the clinical picture, [[fasting]] VIP [[blood plasma|plasma]] level may confirm the diagnosis, and [[CT scan]] and [[somatostatin]] receptor [[scintigraphy]] are used to localise the [[tumor]], which is usually [[metastasis|metastatic]] at presentation.{{cn|date=March 2019}}
* Electrolyte disturbances including [[hypokalemia]], [[hypochlorhydria]], and [[metabolic acidosis]]
* Systemic vasodilation leading to [[flushing]] and [[hypotension]]
* Possible [[hypercalcemia]] and [[hyperglycemia]]


Tests include:
== Clinical Presentation ==
* Blood chemistry tests (basic or comprehensive metabolic panel)
Patients with VIPoma typically present with:
* CT scan of the abdomen
* MRI of the abdomen
* Stool examination for cause of diarrhea and electrolyte levels
* Vasoactive intestinal peptide (VIP) level in the blood{{cn|date=March 2019}}


==Treatment==
* Severe, persistent watery diarrhea (even during fasting)
The first goal of treatment is to correct dehydration. Fluids are often given through a vein (intravenous fluids) to replace fluids lost in diarrhea.
* [[Dehydration]]
* Muscle weakness, cramping
* Weight loss
* [[Facial flushing]]
* [[Hypotension]]
* Nausea or vomiting (less common)


The next goal is to slow the diarrhea. Some medications can help control diarrhea. [[Octreotide]], which is a human-made form of the natural hormone somatostatin, blocks the action of VIP.
== Diagnosis ==
Diagnosis involves a combination of clinical suspicion and confirmatory tests:


The best chance for a cure is surgery to remove the tumor. If the tumor has not spread to other organs, surgery can often cure the condition.
* '''Laboratory tests:'''
* Elevated serum VIP levels (>75 pg/mL)
* Electrolyte imbalances: low potassium, low bicarbonate
* Metabolic acidosis on [[arterial blood gas]]
* '''Imaging:'''
* [[Computed tomography|CT scan]] or [[magnetic resonance imaging|MRI]] to localize tumor
* [[Somatostatin receptor scintigraphy]] (Octreoscan) or [[68Ga-DOTATATE PET/CT]] to detect somatostatin receptor-positive lesions
* [[Endoscopic ultrasound]] in select cases


For metastatic disease, [[peptide receptor radionuclide therapy]] (PRRT) can be highly effective. This treatment involves attaching a radionuclide (Lutetium-177 or Yttrium-90) to a somatostatin analogue (octreotate or octreotide). This is a novel way to deliver high doses of beta radiation to kill tumours.
== Treatment ==
Treatment strategies include:


Some people seem to respond to a combination chemo called capecitabine  and temozolomide but there is no report that it totally cured people from vipoma.{{cn|date=March 2019}}
=== Medical Management ===
* '''[[Octreotide]]''' or '''[[Lanreotide]]''' – long-acting [[somatostatin analogs]] that inhibit VIP secretion and reduce diarrhea
* '''IV fluid and electrolyte replacement''' – to manage dehydration and correct imbalances
* '''Antidiarrheal agents''' – adjunctive, but generally not effective alone


==Prognosis==
=== Surgical Management ===
* '''Surgical resection''' – curative if the tumor is localized
* '''Debulking surgery''' – may help in metastatic cases to control hormone secretion


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.{{cn|date=March 2019}}
=== Advanced/Metastatic Disease ===
* '''Targeted therapy''' – e.g., everolimus, sunitinib
* '''Peptide receptor radionuclide therapy''' (PRRT) for somatostatin receptor-positive tumors
* '''Hepatic artery embolization''' or ablation for liver metastases
 
== Prognosis ==
The prognosis of VIPoma depends on tumor size, location, and metastatic spread. Localized VIPomas have a good prognosis with surgical resection, while metastatic VIPomas have a poorer outcome. However, symptom control with somatostatin analogs can significantly improve quality of life.
 
== Epidemiology ==
VIPomas are extremely rare, with an estimated incidence of approximately 1 per 10 million people per year. They typically occur in adults aged 30–50, with equal incidence in males and females. They may be associated with [[multiple endocrine neoplasia type 1]] (MEN1).
 
== See Also ==
* [[Neuroendocrine tumor]]
* [[Somatostatin]]
* [[Multiple endocrine neoplasia]]
* [[Carcinoid syndrome]]
* [[Pancreatic cancer]]


==References==
{{reflist}}
* Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease . 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 32.
* [http://health.nytimes.com/health/guides/disease/vipoma/overview.html National Cancer Institute. Islet cell tumors (pancreatic) treatment PDQ. Updated October 31, 2008.]
== External links ==
== External links ==
{{Medical resources
{{Medical resources
| DiseasesDB     = 13877
| DiseasesDB = 13877
| ICD10           = {{ICD10|C|25|4|c|15}} or {{ICD10|E|16|8|e|15}}
| ICD10 = {{ICD10|C|25|4|c|15}} or {{ICD10|E|16|8|e|15}}
| ICD9           =  
| ICD9 =
| ICDO           = {{ICDO|8155|3}}
| ICDO = {{ICDO|8155|3}}
| OMIM           =  
| OMIM =
| MedlinePlus     = 000228
| MedlinePlus = 000228
| eMedicineSubj   = med
| eMedicineSubj = med
| eMedicineTopic = 2379
| eMedicineTopic = 2379
| eMedicine_mult = {{eMedicine2|med|2399}} {{eMedicine2|ped|2428}}
| eMedicine_mult = {{eMedicine2|med|2399}} {{eMedicine2|ped|2428}}
| MeshID         = D003969
| MeshID = D003969
| SNOMED CT       = 447643008
| SNOMED CT = 447643008
| Orphanet       = 97282
| Orphanet = 97282
}}
}}
{{Gastrointestinal diseases}}
{{Oncology}}
{{Tumor morphology}}
{{Tumor morphology}}
{{Endocrine gland neoplasia}}
{{Endocrine gland neoplasia}}
[[Category:Endocrine neoplasia]]
[[Category:Endocrine neoplasia]]
[[Category:Rare cancers]]
[[Category:Rare cancers]]
[[Category:Syndromes]]
[[Category:Syndromes]]
{{stb}}
{{No image}}

Latest revision as of 03:20, 3 April 2025

A rare neuroendocrine tumor that causes watery diarrhea and electrolyte imbalance


VIPoma
Synonyms Verner–Morrison syndrome, Pancreatic cholera, WDHA syndrome (Watery Diarrhea, Hypokalemia, and Achlorhydria)
Pronounce
Specialty N/A
Symptoms Profuse watery diarrhea, dehydration, flushing, cramping, weakness
Complications Hypokalemia, achlorhydria, metabolic acidosis, renal failure, malnutrition
Onset Typically adults aged 30–50
Duration Chronic unless treated
Types Pancreatic or extrapancreatic neuroendocrine tumor
Causes Neoplasm of VIP-secreting cells
Risks Multiple endocrine neoplasia type 1
Diagnosis Plasma VIP level, imaging (CT scan, MRI, somatostatin receptor scintigraphy)
Differential diagnosis Cholera, Carcinoid syndrome, laxative abuse, Zollinger–Ellison syndrome
Prevention None known
Treatment Surgical resection, somatostatin analogs, supportive care
Medication Octreotide, Lanreotide, electrolyte replacement
Prognosis Variable; often poor in metastatic cases
Frequency ~1 per 10 million annually
Deaths Associated with complications of metastatic disease or severe dehydration


VIPoma is a rare type of neuroendocrine tumor that secretes excessive amounts of vasoactive intestinal peptide (VIP), a hormone involved in regulating intestinal water and electrolyte secretion. The excessive VIP leads to a distinct clinical syndrome characterized by profound watery diarrhea, hypokalemia (low potassium), and achlorhydria (lack of stomach acid), collectively known as WDHA syndrome or Verner–Morrison syndrome. It is also colloquially referred to as pancreatic cholera due to the similarity of symptoms to cholera infection.

Pathophysiology[edit]

VIPomas most commonly originate from the pancreas, particularly the non-β islet cells, although rare cases have been found in extrapancreatic sites such as the bronchus, adrenal gland, or colon. Approximately 50–75% of VIPomas are malignant at the time of diagnosis, frequently with metastasis to the liver or lymph nodes.

The excessive VIP causes increased intestinal secretion of water and electrolytes, reduced gastric acid secretion, and smooth muscle relaxation, resulting in:

Clinical Presentation[edit]

Patients with VIPoma typically present with:

Diagnosis[edit]

Diagnosis involves a combination of clinical suspicion and confirmatory tests:

Treatment[edit]

Treatment strategies include:

Medical Management[edit]

  • Octreotide or Lanreotide – long-acting somatostatin analogs that inhibit VIP secretion and reduce diarrhea
  • IV fluid and electrolyte replacement – to manage dehydration and correct imbalances
  • Antidiarrheal agents – adjunctive, but generally not effective alone

Surgical Management[edit]

  • Surgical resection – curative if the tumor is localized
  • Debulking surgery – may help in metastatic cases to control hormone secretion

Advanced/Metastatic Disease[edit]

  • Targeted therapy – e.g., everolimus, sunitinib
  • Peptide receptor radionuclide therapy (PRRT) for somatostatin receptor-positive tumors
  • Hepatic artery embolization or ablation for liver metastases

Prognosis[edit]

The prognosis of VIPoma depends on tumor size, location, and metastatic spread. Localized VIPomas have a good prognosis with surgical resection, while metastatic VIPomas have a poorer outcome. However, symptom control with somatostatin analogs can significantly improve quality of life.

Epidemiology[edit]

VIPomas are extremely rare, with an estimated incidence of approximately 1 per 10 million people per year. They typically occur in adults aged 30–50, with equal incidence in males and females. They may be associated with multiple endocrine neoplasia type 1 (MEN1).

See Also[edit]

External links[edit]

Gastrointestinal diseases
General
Upper GI Tract
Lower GI Tract
Accessory organs
Related topics
This gastrointestinal diseases related article is a stub.




Oncology
Basics
Diagnostics
Treatments
Types of Cancers
Related Fields
This oncology-related article is a stub.



Glandular and epithelial cancer
Epithelium
Glands
Adenomas/
adenocarcinomas
Adnexal and
skin appendage
Cystic, mucinous,
and serous
Ductal, lobular,
and medullary



Tumours of endocrine glands
Pancreas
Pituitary
Thyroid
Adrenal tumor
Parathyroid
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