VIPoma overview: Difference between revisions
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{{VIPoma}} | {{VIPoma}} | ||
{{CMG}}{{AE}}{{PSD}} | {{CMG}}{{AE}}{{MSI}}{{PSD}}{{Homa}} | ||
==Overview== | ==Overview== | ||
VIPoma is a [[rare]] [[tumor]] of the non-[[beta cells]] of the [[pancreas]] that results in the overproduction of the [[hormone]] [[vasoactive intestinal peptide]] ([[VIP]]). On [[histopathological]] [[analysis]], composition of uniform, small to intermediate-sized [[Cells (biology)|cells]] in clusters, nests, and [[Trabecular tissue|trabecular]] [[growth]] [[Pattern|patterns]] with hyperchromatic [[nuclei]] and scant [[cytoplasm]] are seen. VIPoma must be [[Differentiate|differentiated]] from ganglioneuroblastoma, [[ganglioneuroma]], factitious [[diarrhea]], [[Bile salts|bile salt]] [[enteropathy]], [[rectal]] vilous [[adenomas]], and [[laxative abuse]]. The [[incidence]] VIPoma is approximately 0.01 per 100,000 individuals worldwide, and [[females]] are more commonly [[Affect|affected]] than [[males]]. If left untreated, [[patients]] with VIPoma may progress to [[Development|develop]] [[watery diarrhea]], [[abdominal pain]], [[bloating]], [[nausea]], [[vomiting]], [[skin rash]], [[backache]], [[flushing]], and [[lethargy]]. Common [[complications]] of VIPoma include [[metastasis]], [[cardiac arrest]] from [[low blood potassium]] level, and [[dehydration]]. The presence of [[metastasis]] is associated with a particularly poor [[prognosis]], with a 5 year [[survival rate]] of 60%. The [[hallmark]] of VIPoma is [[watery diarrhea]]. A positive [[History and Physical examination|history]] of [[abdominal pain]], [[weight loss]], [[numbness]], and [[weakness]] is suggestive of VIPoma. Common [[physical examination]] findings of VIPoma include [[tachycardia]], [[rash]], [[facial flushing]], [[abdominal tenderness]], and [[abdominal distention]]. [[Laboratory]] tests used in the [[diagnosis]] of VIPoma include [[serum]] [[vasoactive intestinal polypeptide]] ([[VIP]]) levels, [[Basal (medicine)|basal]] [[gastric acid]] output, and basic [[metabolic]] pannel for [[potassium]], [[bicarbonate]], [[magnesium]], and [[calcium]] levels. On [[CT scan]] VIPoma is characterized by hypervascularity with [[diffuse]] multiple [[metastatic]] [[Nodule (medicine)|nodulation]]. [[Abdominal]] [[MRI]] is helpful in the [[diagnosis]] of VIPoma which is characterized by a [[mass]] that is hypointense on [[T1]]-weighted and hyperintense on T2-weighted [[MRI]]. Initial treatment in [[patient]] with VIPoma is prompt replacement of [[fluid]] and [[electrolyte]] losses, [[steroids]] may be used to provide [[symptomatic]] relief. [[Surgery]] is the mainstay of [[Therapy|treatment]]. | |||
The | |||
==Historical Perspective== | ==Historical Perspective== | ||
VIPoma was first described in 1958 by Verner and Morrison. | VIPoma which is also known as [[Verner-Morrison syndrome]] was first described in 1958 by Verner and Morrison. | ||
==Pathophysiology== | ==Pathophysiology== | ||
A VIPoma is a rare tumor of the non-beta cells of the pancreas that results in the overproduction of the hormone vasoactive intestinal peptide (VIP). On microscopic histopathological analysis, findings of VIPoma are composition of uniform, small to intermediate-sized cells in clusters, nests, and trabecular growth patterns with hyperchromatic nuclei and scant cytoplasm. | A VIPoma is a [[rare]] [[tumor]] of the non-[[Beta cell|beta cells]] of the [[pancreas]] that results in the overproduction of the [[hormone]] [[vasoactive intestinal peptide]] ([[Vasoactive intestinal peptide|VIP]]). On [[microscopic]][[histopathological]] [[analysis]], findings of VIPoma are composition of uniform, small to intermediate-sized [[Cells (biology)|cells]] in clusters, nests, and [[Trabecular tissue|trabecular]] [[growth]] [[Pattern|patterns]] with hyperchromatic [[nuclei]] and scant [[cytoplasm]]. | ||
==Causes== | ==Causes== | ||
The [[Causes|cause]] of VIPoma has not been identified. | |||
== | ==Differentiating VIPoma From Other Diseases== | ||
VIPoma must be differentiated from ganglioneuroblastoma, [[ganglioneuroma]], factitious [[diarrhea]], | VIPoma must be differentiated from ganglioneuroblastoma, [[ganglioneuroma]], factitious [[diarrhea]], [[bile salt]] [[enteropathy]], [[rectal]] vilous [[adenomas]], and [[laxative abuse]]. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The incidence VIPoma is approximately 0.01 per 100,000 individuals worldwide. | The annual [[incidence]] of VIPoma is approximately 0.01 per 100,000 (approx. 1 in 10 million) individuals worldwide. [[Female]] are more commonly affected by VIPoma than [[male]]. The [[incidence]] of VIPoma increases with [[age]], the [[median]] [[age]] at [[diagnosis]] in [[Adult|adults]] is 50 years. VIPoma in [[children]] is usually [[Diagnose|diagnosed]] between [[age]] 2 to 4. | ||
==Risk Factors== | ==Risk Factors== | ||
The most | The most important [[risk factor]] in the [[development]] of VIPoma is a positive [[family history]] of [[multiple endocrine neoplasia type 1]]. | ||
==Screening== | ==Screening== | ||
According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for VIPoma. | According to the U.S. Preventive Service Task Force ([[USPSTF guidelines|USPSTF]]), there is insufficient [[evidence]] to recommend routine [[Screening (medicine)|screening]] for VIPoma. | ||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
If left untreated, patients with VIPoma may progress to develop | If left untreated, [[patients]] with VIPoma may progress to [[Development|develop]] [[watery diarrhea]], [[abdominal pain]], [[bloating]], [[nausea]], [[vomiting]], [[skin rash]], [[backache]], [[flushing]], and [[lethargy]]. Common [[complications]] of VIPoma include [[metastasis]], [[cardiac arrest]] from [[low blood potassium]] level, and [[dehydration]]. The presence of [[metastasis]] is associated with a particularly poor [[prognosis]] among [[patients]] with VIPoma, with a 5 year [[survival rate]] of 60%. | ||
==Diagnosis== | |||
The | ===Diagnostic Study of Choice=== | ||
The [[diagnostic study of choice]] for Vipoma is the [[measurement]] of [[serum]] [[Vasoactive intestinal peptide|vasoactive intestinal polypeptide]] ([[Vasoactive intestinal peptide|VIP]]) [[concentration]]. | |||
==Other Imaging Findings== | ===History and Symptoms=== | ||
Other imaging studies for VIPoma include [[somatostatin]] | The [[hallmark]] of Vipoma is [[watery diarrhea]]. A positive [[History and Physical examination|history]] of [[abdominal pain]], [[weight loss]], [[numbness]], and [[weakness]] is suggestive of VIPoma . The most common [[symptoms]] of VIPoma include [[watery diarrhea]] like [[cholera]], [[Dehydration|dehydration,]] [[lethargy]], [[muscle weakness]], [[weight loss]], [[numbness]], and [[flushing]]. | ||
==Medical Therapy== | ===Physical Examination=== | ||
Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte | Common [[physical examination]] findings of VIPoma include [[tachycardia]], [[rash]], [[facial flushing]], [[abdominal tenderness]], [[muscle weakness]], and [[abdominal distention]]. | ||
==Surgery== | ===Laboratory Findings=== | ||
Surgery is the mainstay of treatment for VIPoma. | [[Laboratory]] [[Test|tests]] used in the [[diagnosis]] of VIPoma include [[serum]] [[Vasoactive intestinal peptide|vasoactive intestinal polypeptide]] ([[VIP]]) levels, basal [[gastric acid]] output, and [[Comprehensive metabolic panel|CMP]] for [[potassium]], [[bicarbonate]], [[magnesium]], and [[calcium]] levels. | ||
==Primary Prevention== | ===Electrocardiogram=== | ||
There | There are no [[ECG]] findings associated with VIPoma. | ||
==Secondary Prevention== | |||
===X-ray=== | |||
There are no [[x-ray]] findings associated with VIPoma. | |||
===CT=== | |||
On [[CT scan]] VIPoma is characterized by hypervascularity with [[diffuse]] multiple [[metastatic]] [[nodulation]]. [[Computed tomography|CT scan]] are highly accurate for [[tumor]] localization of primary [[neuroendocrine]] [[pancreatic tumor]]. Since most of them are more than 3cm in size at the time of presentation. [[Sensitivity]] of [[contrast]] enhanced [[Computed tomography|CT]] for VIPoma approaches 100%. | |||
===MRI=== | |||
[[Abdominal]] [[MRI]] is helpful in the [[diagnosis]] of VIPoma. On [[abdominal]] [[MRI]], VIPoma is characterized by a [[mass]] which is hypointense on [[T1]]-weighted [[MRI]] and hyperintense on T2-weighted [[MRI]]. | |||
===Echocardiography or Ultrasound=== | |||
Endoscopic [[ultrasound]] may be helpful in the [[diagnosis]] of VIPoma. Finding on [[ultrasound]] suggestive of VIPoma is hypoechoic [[tumor]] in the [[distal]] part of [[pancreas]]. | |||
===Other Imaging Findings=== | |||
Other [[imaging studies]] for VIPoma include [[somatostatin receptor]] [[Nuclear medicine|scintigraphy]] and [[Positron emission tomography|PET scan]] using radiolabeled [[somatostatin]] [[Analog (chemistry)|analogs]]. | |||
===Other Diagnostic Studies=== | |||
Other [[diagnostic]] studies for VIPoma include [[immunohistochemical staining]] [[test]], which demonstrates [[staining]] for [[Marker|markers]] such as [[chromogranin A]], [[Cytokeratin|cytokeratin 19]], [[synaptophysin]], [[Ki-67]], [[Enolase|neuron specific enolase]], PGP 9.5. | |||
==Treatment== | |||
===Medical Therapy=== | |||
Initial treatment in [[patient]] with VIPoma is [[prompt]] [[Fluid replacement therapy|replacement of fluid]] and correction of [[electrolyte imbalance]] and [[Acid-base disturbances|acid-base disturbance]]. [[Sandostatin|Somatostatin]] analogues like short acting [[octreotide]] is useful for controlling [[diarrhea]] by blocking the release of [[Vasoactive intestinal peptide|VIP]]. [[Octreotide]] is later replaced by longer acting depot preparation of [[somatostatin]] analogues like [[sandostatin]] or [[lanreotide]]. | |||
=== Interventions === | |||
The mainstay of treatment for VIPoma is [[surgery]]. [[Hepatic artery]] [[embolization]] or transcatheter [[chemoembolization]] with [[doxorubicin]] or [[cisplatin]] is usually reserved for patients with [[liver]] [[metastases]]. Moreover, in [[patients]] with [[liver]] [[metastases]] less than 3 cm [[radiofrequency ablation]] and [[cryoablation]] can be used. | |||
===Surgery=== | |||
[[Surgery]] is the [[mainstay]] of treatment for VIPoma. [[Surgery]] should be considered after initial [[symptomatic]] management of VIPoma [[inoperable]] cases. Complete [[surgical resection]] of the [[tumor]] is the only [[Cure|curative]] treatment for VIPoma. If the [[tumor]] cannot be removed completely, [[Surgery|surgical]] [[debulking]] may have [[Palliative therapy|palliative]] effect for [[control]] of [[hormonal]] [[symptoms]]. | |||
===Primary Prevention=== | |||
There are no established [[Measurement|measures]] for the [[primary prevention]] of VIPoma. | |||
===Secondary Prevention=== | |||
Effective [[Measurement|measures]] for the [[secondary prevention]] of VIPoma include [[History and Physical examination|history and physical examination]], [[serum]] [[Vasoactive intestinal peptide|VIP]] levels and [[Indication (medicine)|indicated]] [[Marker|markers]], and multi-phasic [[Computed tomography|CT scan]] or [[MRI]]. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
[[Category:Medicine]] | |||
[[Category:Oncology]] | |||
[[Category:Up-To-Date]] | |||
[[Category:Endocrinology]] | |||
[[Category:Gastroenterology]] | |||
Latest revision as of 00:40, 30 July 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Madhu Sigdel M.B.B.S.[2]Parminder Dhingra, M.D. [3] Homa Najafi, M.D.[4]
Overview
VIPoma is a rare tumor of the non-beta cells of the pancreas that results in the overproduction of the hormone vasoactive intestinal peptide (VIP). On histopathological analysis, composition of uniform, small to intermediate-sized cells in clusters, nests, and trabecular growth patterns with hyperchromatic nuclei and scant cytoplasm are seen. VIPoma must be differentiated from ganglioneuroblastoma, ganglioneuroma, factitious diarrhea, bile salt enteropathy, rectal vilous adenomas, and laxative abuse. The incidence VIPoma is approximately 0.01 per 100,000 individuals worldwide, and females are more commonly affected than males. If left untreated, patients with VIPoma may progress to develop watery diarrhea, abdominal pain, bloating, nausea, vomiting, skin rash, backache, flushing, and lethargy. Common complications of VIPoma include metastasis, cardiac arrest from low blood potassium level, and dehydration. The presence of metastasis is associated with a particularly poor prognosis, with a 5 year survival rate of 60%. The hallmark of VIPoma is watery diarrhea. A positive history of abdominal pain, weight loss, numbness, and weakness is suggestive of VIPoma. Common physical examination findings of VIPoma include tachycardia, rash, facial flushing, abdominal tenderness, and abdominal distention. Laboratory tests used in the diagnosis of VIPoma include serum vasoactive intestinal polypeptide (VIP) levels, basal gastric acid output, and basic metabolic pannel for potassium, bicarbonate, magnesium, and calcium levels. On CT scan VIPoma is characterized by hypervascularity with diffuse multiple metastatic nodulation. Abdominal MRI is helpful in the diagnosis of VIPoma which is characterized by a mass that is hypointense on T1-weighted and hyperintense on T2-weighted MRI. Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte losses, steroids may be used to provide symptomatic relief. Surgery is the mainstay of treatment.
Historical Perspective
VIPoma which is also known as Verner-Morrison syndrome was first described in 1958 by Verner and Morrison.
Pathophysiology
A VIPoma is a rare tumor of the non-beta cells of the pancreas that results in the overproduction of the hormone vasoactive intestinal peptide (VIP). On microscopichistopathological analysis, findings of VIPoma are composition of uniform, small to intermediate-sized cells in clusters, nests, and trabecular growth patterns with hyperchromatic nuclei and scant cytoplasm.
Causes
The cause of VIPoma has not been identified.
Differentiating VIPoma From Other Diseases
VIPoma must be differentiated from ganglioneuroblastoma, ganglioneuroma, factitious diarrhea, bile salt enteropathy, rectal vilous adenomas, and laxative abuse.
Epidemiology and Demographics
The annual incidence of VIPoma is approximately 0.01 per 100,000 (approx. 1 in 10 million) individuals worldwide. Female are more commonly affected by VIPoma than male. The incidence of VIPoma increases with age, the median age at diagnosis in adults is 50 years. VIPoma in children is usually diagnosed between age 2 to 4.
Risk Factors
The most important risk factor in the development of VIPoma is a positive family history of multiple endocrine neoplasia type 1.
Screening
According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for VIPoma.
Natural History, Complications and Prognosis
If left untreated, patients with VIPoma may progress to develop watery diarrhea, abdominal pain, bloating, nausea, vomiting, skin rash, backache, flushing, and lethargy. Common complications of VIPoma include metastasis, cardiac arrest from low blood potassium level, and dehydration. The presence of metastasis is associated with a particularly poor prognosis among patients with VIPoma, with a 5 year survival rate of 60%.
Diagnosis
Diagnostic Study of Choice
The diagnostic study of choice for Vipoma is the measurement of serum vasoactive intestinal polypeptide (VIP) concentration.
History and Symptoms
The hallmark of Vipoma is watery diarrhea. A positive history of abdominal pain, weight loss, numbness, and weakness is suggestive of VIPoma . The most common symptoms of VIPoma include watery diarrhea like cholera, dehydration, lethargy, muscle weakness, weight loss, numbness, and flushing.
Physical Examination
Common physical examination findings of VIPoma include tachycardia, rash, facial flushing, abdominal tenderness, muscle weakness, and abdominal distention.
Laboratory Findings
Laboratory tests used in the diagnosis of VIPoma include serum vasoactive intestinal polypeptide (VIP) levels, basal gastric acid output, and CMP for potassium, bicarbonate, magnesium, and calcium levels.
Electrocardiogram
There are no ECG findings associated with VIPoma.
X-ray
There are no x-ray findings associated with VIPoma.
CT
On CT scan VIPoma is characterized by hypervascularity with diffuse multiple metastatic nodulation. CT scan are highly accurate for tumor localization of primary neuroendocrine pancreatic tumor. Since most of them are more than 3cm in size at the time of presentation. Sensitivity of contrast enhanced CT for VIPoma approaches 100%.
MRI
Abdominal MRI is helpful in the diagnosis of VIPoma. On abdominal MRI, VIPoma is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.
Echocardiography or Ultrasound
Endoscopic ultrasound may be helpful in the diagnosis of VIPoma. Finding on ultrasound suggestive of VIPoma is hypoechoic tumor in the distal part of pancreas.
Other Imaging Findings
Other imaging studies for VIPoma include somatostatin receptor scintigraphy and PET scan using radiolabeled somatostatin analogs.
Other Diagnostic Studies
Other diagnostic studies for VIPoma include immunohistochemical staining test, which demonstrates staining for markers such as chromogranin A, cytokeratin 19, synaptophysin, Ki-67, neuron specific enolase, PGP 9.5.
Treatment
Medical Therapy
Initial treatment in patient with VIPoma is prompt replacement of fluid and correction of electrolyte imbalance and acid-base disturbance. Somatostatin analogues like short acting octreotide is useful for controlling diarrhea by blocking the release of VIP. Octreotide is later replaced by longer acting depot preparation of somatostatin analogues like sandostatin or lanreotide.
Interventions
The mainstay of treatment for VIPoma is surgery. Hepatic artery embolization or transcatheter chemoembolization with doxorubicin or cisplatin is usually reserved for patients with liver metastases. Moreover, in patients with liver metastases less than 3 cm radiofrequency ablation and cryoablation can be used.
Surgery
Surgery is the mainstay of treatment for VIPoma. Surgery should be considered after initial symptomatic management of VIPoma inoperable cases. Complete surgical resection of the tumor is the only curative treatment for VIPoma. If the tumor cannot be removed completely, surgical debulking may have palliative effect for control of hormonal symptoms.
Primary Prevention
There are no established measures for the primary prevention of VIPoma.
Secondary Prevention
Effective measures for the secondary prevention of VIPoma include history and physical examination, serum VIP levels and indicated markers, and multi-phasic CT scan or MRI.
References