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Malignant Vipoma

MedGen UID:
363986
•Concept ID:
C1881600
•
Neoplastic Process
Synonym: Vipoma, malignant
SNOMED CT: Vipoma, malignant (31131002)

Definition

An aggressive neuroendocrine tumor located in the pancreas or small intestine. It is composed of cells containing vasoactive intestinal peptide. It is associated with watery diarrhea, hypokalemia, and hypochlorhydria or achlorhydria. It displays vascular invasion and metastasizes to other anatomic sites. [from NCI]

Professional guidelines

PubMed

Treatment options of metastatic and nonmetastatic VIPoma: a review.
Azizian A, König A, Ghadimi M
Langenbecks Arch Surg 2022 Nov;407(7):2629-2636. Epub 2022 Aug 5 doi: 10.1007/s00423-022-02620-7. PMID: 35931878Free PMC Article
Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.
Ito T, Jensen RT
Expert Opin Pharmacother 2021 Apr;22(6):685-693. Epub 2020 Nov 11 doi: 10.1080/14656566.2020.1845651. PMID: 33131345Free PMC Article
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Recent clinical studies

Etiology

Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present.
Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP
J Pediatr Surg 2002 Jun;37(6):887-92. doi: 10.1053/jpsu.2002.32897. PMID: 12037756
Pancreaticoduodenectomy for selected pancreatic endocrine tumors.
Udelsman R, Yeo CJ, Hruban RH, Pitt HA, Niederhuber JE, Coleman J, Cameron JL
Surg Gynecol Obstet 1993 Sep;177(3):269-78. PMID: 8102815

Diagnosis

Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present.
Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP
J Pediatr Surg 2002 Jun;37(6):887-92. doi: 10.1053/jpsu.2002.32897. PMID: 12037756

Therapy

Pancreaticoduodenectomy for selected pancreatic endocrine tumors.
Udelsman R, Yeo CJ, Hruban RH, Pitt HA, Niederhuber JE, Coleman J, Cameron JL
Surg Gynecol Obstet 1993 Sep;177(3):269-78. PMID: 8102815

Prognosis

Pancreaticoduodenectomy for selected pancreatic endocrine tumors.
Udelsman R, Yeo CJ, Hruban RH, Pitt HA, Niederhuber JE, Coleman J, Cameron JL
Surg Gynecol Obstet 1993 Sep;177(3):269-78. PMID: 8102815

Clinical prediction guides

Somatic mutations of multiple endocrine neoplasia type 1 gene in the sporadic endocrine tumors.
Shan L, Nakamura Y, Nakamura M, Yokoi T, Tsujimoto M, Arima R, Kameya T, Kakudo K
Lab Invest 1998 Apr;78(4):471-5. PMID: 9564891

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