VIPoma

VIPoma is an extremely rare type of pancreatic neuroendocrine tumor (see this term) that secretes vasoactive intestinal polypeptide (VIP) leading to the manifestations of watery diarrhea, hypokalemia and achlorhydia or hypochhlorhydia (known as WDHA syndrome).



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Narrow down the case reports



Total: 76 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(54.2%)
6251696
Watery diarrhea, hypokalemia and achlorhydria syndrome. Morphological and immunohistological study.
Kasajima T, Shinzawa H, Matsuda M, Terashima K, Imai Y.
Acta Pathol Jpn. 1980;30(4):639-50.
Jaundice Anemia Dehydration
VIP
Achlorhydria Adult Cytoplasmic Granules Diarrhea Homo sapiens Hypokalemia Islet Cell Adenoma Male Pancreatic Neoplasm
2
(28.3%)
21737909
Rheumatoid arthritis associated with the use of Sandostatin LAR depot in a patient with pancreatic neuroendocrine tumor. An association or a coincidence? The first case report.
Saif MW.
JOP. 2011;12(4):425-8.
Rheumatoid arthritis Hypoglycemia Pancreatic pseudocyst Flushing
VIP
Antineoplastic Agents, Hormonal Delayed-Action Preparations Females Homo sapiens Incidence Middle Aged Neuroendocrine Tumors Pancreatic Neoplasm Rheumatoid Arthritis
3
(26.9%)
19101728
Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl.
Zhang WQ, Liu JF, Zhao J, Zhao SY, Xue Y.
Eur J Pediatr. 2009;168(7):859-62.
Episodic hypertension Fever Vascular dilatation Flushing
VIP
Achlorhydria Child, Preschool Diarrhea Females Ganglioneuroma Homo sapiens Hypokalemia Thoracic Neoplasms X-Ray Computed Tomography
4
(26.3%)
30510956
(6264997)
Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature.
Hu X, Cao W, Zhao M.
World J Clin Cases. 2018;6(14):862-868.
Hypertension Diarrhea Hypokalemia Flushing
SSTR2 VIP
5
(21.6%)
25081061
(4122777)
A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma.
Jiang J, Zhang L, Wu Z, Ai Z, Hou Y, Lu Z, Gao X.
BMC Cancer. 2014;14:553.
Metabolic acidosis Diarrhea
Achlorhydria Adrenal Gland Neoplasms Diarrhea Disease-Free Survival Homo sapiens Hypokalemia Male Middle Aged Pheochromocytoma
5
(21.6%)
20558361
[Verner-Morrison syndrome: a case study].
Halaszlaki C, Horvath H, Kiss L, Takacs I, Speer G, Nagy Z, Winternitz T, Dabasi G, Zalatnai A, Patocs A, Lakatos P.
Orv Hetil. 2010;151(27):1111-4.
Dehydration Diarrhea
VIP
Achlorhydria Biomarkers, Tumor Diarrhea Echo Endoscopy Females Homo sapiens Hypokalemia Immunohistochemistry Magnetic Resonance Imaging Multiple Endocrine Neoplasia Type 1 Pancreatic Neoplasm Vipoma X-Ray Computed Tomography
5
(21.6%)
19488018
Hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vipoma.
Kibria R, Ahmed S, Ali SA, Barde CJ.
South Med J. 2009;102(7):761-4.
Diarrhea Hypokalemia
VIP
Achlorhydria Adult Diarrhea Homo sapiens Hypokalemia Male Pancreatic Neoplasm Rhabdomyolysis Syndrome Vipoma
5
(21.6%)
19184565
VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome.
Nakayama S, Yokote T, Kobayashi K, Hirata Y, Hiraiwa T, Komoto I, Miyakoshi K, Yamakawa Y, Takubo T, Tsuji M, Imamura M, Hanafusa T.
Endocrine. 2009;35(2):143-6.
Diarrhea Hypokalemia
Achlorhydria Diarrhea Females Gene Expression Homo sapiens Hypokalemia Immunohistochemistry Indium Radioisotopes Isotope Labeling Magnetic Resonance Imaging Pancreatic Neoplasm Receptors, Vasoactive Intestinal Peptide Syndrome Vipoma X-Ray Computed Tomography
5
(21.6%)
17729424
Watery diarrhea, hypokalemia and achlorhydria syndrome due to an adrenal pheochromocytoma.
Ikuta S, Yasui C, Kawanaka M, Aihara T, Yoshie H, Yanagi H, Mitsunobu M, Sugihara A, Yamanaka N.
World J Gastroenterol. 2007;13(34):4649-52.
Diarrhea Hypokalemia
VIP
Achlorhydria Adrenal Gland Neoplasms Adrenalectomy Catecholamines Diarrhea Females Homo sapiens Hypokalemia Incidental Findings Middle Aged Pheochromocytoma Vipoma X-Ray Computed Tomography
5
(21.6%)
16027147
Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1.
Onozawa M, Fukuhara T, Minoguchi M, Takahata M, Yamamoto Y, Miyake T, Kanagawa K, Kanda M, Maekawa I.
Jpn J Clin Oncol. 2005;35(9):559-63.
Muscle weakness Diarrhea Hypokalemia
NF1 VIP
Achlorhydria Adrenal Gland Neoplasms Diarrhea Females Homo sapiens Hypokalemia Middle Aged Neurofibromatosis 1 Pheochromocytoma Radionuclide Imaging Radiopharmaceuticals Rhabdomyolysis Syndrome X-Ray Computed Tomography
        

Phenotype(s) retrieved from Orphanet

    Total: 41

HPO ID Term Frequency
HP:0002894 Neoplasm of the pancreas Very frequent (99-80%)
HP:0002900 Hypokalemia Very frequent (99-80%)
HP:0005208 Secretory diarrhea Very frequent (99-80%)
HP:0000819 Diabetes mellitus Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0001895 Normochromic anemia Frequent (79-30%)
HP:0001944 Dehydration Frequent (79-30%)
HP:0002017 Nausea and vomiting Frequent (79-30%)
HP:0002024 Malabsorption Frequent (79-30%)
HP:0002039 Anorexia Frequent (79-30%)
HP:0002240 Hepatomegaly Frequent (79-30%)
HP:0002574 Episodic abdominal pain Frequent (79-30%)
HP:0003072 Hypercalcemia Frequent (79-30%)
HP:0003324 Generalized muscle weakness Frequent (79-30%)
HP:0003394 Muscle spasm Frequent (79-30%)
HP:0004396 Poor appetite Frequent (79-30%)
HP:0010783 Erythema Frequent (79-30%)
HP:0012432 Chronic fatigue Frequent (79-30%)
HP:0001046 Intermittent jaundice Occasional (29-5%)
HP:0001406 Intrahepatic cholestasis Occasional (29-5%)
HP:0001438 Abnormality of abdomen morphology Occasional (29-5%)
HP:0001541 Ascites Occasional (29-5%)
HP:0012334 Extrahepatic cholestasis Occasional (29-5%)
HP:0030895 Abnormal gastrointestinal motility Occasional (29-5%)
HP:0000820 Abnormality of the thyroid gland Very rare (4-1%)
HP:0000837 Increased circulating gonadotropin level Very rare (4-1%)
HP:0000845 Growth hormone excess Very rare (4-1%)
HP:0000870 Prolactin excess Very rare (4-1%)
HP:0001031 Subcutaneous lipoma Very rare (4-1%)
HP:0001578 None Very rare (4-1%)
HP:0002747 Respiratory insufficiency due to muscle weakness Very rare (4-1%)
HP:0002893 Pituitary adenoma Very rare (4-1%)
HP:0002896 Neoplasm of the liver Very rare (4-1%)
HP:0002897 Parathyroid adenoma Very rare (4-1%)
HP:0003005 Ganglioneuroma Very rare (4-1%)
HP:0003528 Elevated calcitonin Very rare (4-1%)
HP:0006719 Benign gastrointestinal tract tumors Very rare (4-1%)
HP:0006731 Follicular thyroid carcinoma Very rare (4-1%)
HP:0008200 Primary hyperparathyroidism Very rare (4-1%)
HP:0008256 Adrenocortical adenoma Very rare (4-1%)
HP:0002573 Hematochezia Excluded (0%)


Phenotype(s) retrieved from case reports

    Total: 19

HPO ID Term # of case reports
HP:0002014 Diarrhea 22
HP:0002900 Hypokalemia 18
HP:0032448 Achlorhydria 14
HP:0030731 Carcinoma 11
HP:0002666 Pheochromocytoma 5
HP:0005208 Secretory diarrhea 4
HP:0003072 Hypercalcemia 3
HP:0002041 Intractable diarrhea 2
HP:0003201 Rhabdomyolysis 2
HP:0030405 Pancreatic endocrine tumor 2
HP:0001942 Metabolic acidosis 1
HP:0001944 Dehydration 1
HP:0002024 Malabsorption 1
HP:0002897 Parathyroid adenoma 1
HP:0004385 Protracted diarrhea 1
HP:0011106 Hypovolemia 1
HP:0031284 Flushing 1
HP:0040192 APUdoma 1
HP:0100570 Carcinoid tumor 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID