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VIPoma

MedGen UID:: 41532
•Concept ID:: C0011993
•: Neoplastic Process

Synonyms:	Pancreatic VIPoma; Vasoactive intestinal peptide (VIP) tumor; Vasoactive intestinal peptide-producing tumor
SNOMED CT:	VIP-oma - Vasoactive intestinal peptide-secreting tumor (253005002); Vasoactive intestinal peptide-secreting tumor (253005002)
Modes of inheritance:	Not genetically inherited MedGen UID: 988794 •Concept ID: CN307044 • Finding Source: Orphanet clinical entity without genetic inheritance. See: This record Not genetically inherited (Orphanet)

Monarch Initiative:	MONDO:0019960
Orphanet:	ORPHA97282

Definition

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). [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVVIPoma

Disorder of digestive system
- Abnormality of the digestive system
  - Digestive system neoplasm
    - Neoplasm of the gastrointestinal tract
      - Digestive system neuroendocrine neoplasm
        Digestive System Neuroendocrine Tumor
        VIPoma
        Malignant Vipoma
        Malignant Pancreatic Vipoma
        Pancreatic vasoactive intestinal peptide producing tumor
        Small intestinal vasoactive intestinal peptide producing tumor

Follow this link to review classifications for VIPoma in Orphanet.

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: 35931878 Free 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: 33131345 Free PMC Article

Pancreatic neuroendocrine tumors: contemporary diagnosis and management.

Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266

See all (19)

Recent clinical studies

Etiology

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.

Damaskos C, Dimitroulis D, Garmpi A, Antoniou EA, Kouraklis G, Psilopatis I, Mavri M, Diamantis E, Marinos G, Kyriakos G, Farmaki P, Patsouras A, Kontzoglou K, Garmpis N
In Vivo 2023 Nov-Dec;37(6):2402-2408. doi: 10.21873/invivo.13345. PMID: 37905620 Free PMC Article

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: 35931878 Free PMC Article

Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.

Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819

Vasoactive Intestinal Peptide-Secreting Tumors: A Review.

Siddappa PK, Vege SS
Pancreas 2019 Oct;48(9):1119-1125. doi: 10.1097/MPA.0000000000001402. PMID: 31609932

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: 23582916 Free PMC Article

See all (78)

Diagnosis

European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.

Hofland J, Falconi M, Christ E, Castaño JP, Faggiano A, Lamarca A, Perren A, Petrucci S, Prasad V, Ruszniewski P, Thirlwell C, Vullierme MP, Welin S, Bartsch DK
J Neuroendocrinol 2023 Aug;35(8):e13318. Epub 2023 Aug 14 doi: 10.1111/jne.13318. PMID: 37578384

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: 33131345 Free PMC Article

Verner-Morrison syndrome. Literature review.

Belei OA, Heredea ER, Boeriu E, Marcovici TM, Cerbu S, Mărginean O, Iacob ER, Iacob D, Motoc AGM, Boia ES
Rom J Morphol Embryol 2017;58(2):371-376. PMID: 28730220

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: 23582916 Free PMC Article

Pancreatic neuroendocrine tumors.

Batcher E, Madaj P, Gianoukakis AG
Endocr Res 2011;36(1):35-43. doi: 10.3109/07435800.2010.525085. PMID: 21226566

See all (206)

Therapy

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: 35931878 Free 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: 23582916 Free PMC Article

Primary hepatic vipoma.

Ayub A, Zafar M, Abdulkareem A, Ali MA, Lingawi T, Harbi A
Am J Gastroenterol 1993 Jun;88(6):958-61. PMID: 8389095

Future medical prospects for Sandostatin.

Harris AG
Metabolism 1990 Sep;39(9 Suppl 2):180-5. doi: 10.1016/0026-0495(90)90241-4. PMID: 2205787

VIPoma syndrome.

Mekhjian HS, O'Dorisio TM
Semin Oncol 1987 Sep;14(3):282-91. PMID: 2820063

See all (110)

Prognosis

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.

Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging.

Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819

Imaging of Pancreatic-Neuroendocrine Tumours: An Outline of Conventional Radiological Techniques.

Zamir MA, Hakim W, Yusuf S, Thomas R
Curr Radiopharm 2019;12(2):135-155. doi: 10.2174/1874471012666190214165845. PMID: 30767758

Pancreatic neuroendocrine tumors: biology, diagnosis,and treatment.

Ro C, Chai W, Yu VE, Yu R
Chin J Cancer 2013 Jun;32(6):312-24. Epub 2012 Dec 14 doi: 10.5732/cjc.012.10295. PMID: 23237225 Free PMC Article

Solid tumors of the body and tail of the pancreas.

Morgan KA, Adams DB
Surg Clin North Am 2010 Apr;90(2):287-307. doi: 10.1016/j.suc.2009.12.009. PMID: 20362787

See all (76)

Clinical prediction guides

A clinical analysis on functioning pancreatic neuroendocrine tumors (focusing on VIPomas): a single-center experience.

Murakami M, Fujimori N, Matsumoto K, Ohno A, Teramatsu K, Takamatsu Y, Takeno A, Ueda K, Oono T, Ito T, Ogawa Y
Endocr J 2022 Oct 28;69(10):1201-1209. Epub 2022 May 27 doi: 10.1507/endocrj.EJ22-0111. PMID: 35644576

Primary Pancreatic Secretinoma: Further Evidence Supporting Secretin as a Diarrheogenic Hormone.

Chey WY, Frankel WL, Roy S, Datta S, Sen CK, Dillhoff M, Muscarella P, Soergel KH, Tompkins RK, Chang TM, Bradley EL 3rd, Ellison EC
Ann Surg 2017 Aug;266(2):346-352. doi: 10.1097/SLA.0000000000001938. PMID: 27501174 Free PMC Article

Biochemical markers for gastroenteropancreatic neuroendocrine tumours (GEP-NETs).

Kanakis G, Kaltsas G
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):791-802. doi: 10.1016/j.bpg.2012.12.006. PMID: 23582919

Paraneoplastic manifestations in children.

de Graaf JH, Tamminga RY, Kamps WA
Eur J Pediatr 1994 Nov;153(11):784-91. doi: 10.1007/BF01972883. PMID: 7843190

VIPoma syndrome.

Krejs GJ
Am J Med 1987 May 29;82(5B):37-48. doi: 10.1016/0002-9343(87)90425-6. PMID: 3035922

See all (42)