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Malignant Pancreatic Vipoma

MedGen UID:
363581
•Concept ID:
C1881586
•
Neoplastic Process

Definition

A pancreatic neuroendocrine tumor producing vasoactive intestinal peptide (VIP). It is associated with watery diarrhea, hypokalemia, and hypochlorhydria or achlorhydria. It displays vascular invasion and metastasizes to other anatomic sites. One third of cases are metastatic at the time of diagnosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Pancreatic Vipoma

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

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: 37905620Free 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: 35931878Free 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: 23582916Free PMC Article

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
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: 23582916Free PMC Article
VIPoma syndrome.
Krejs GJ
Am J Med 1987 May 29;82(5B):37-48. doi: 10.1016/0002-9343(87)90425-6. PMID: 3035922
Somatostatinomas, PPomas, neurotensinomas.
Vinik AI, Strodel WE, Eckhauser FE, Moattari AR, Lloyd R
Semin Oncol 1987 Sep;14(3):263-81. PMID: 2820062

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: 35931878Free PMC Article
Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.
Dimitriadis GK, Weickert MO, Randeva HS, Kaltsas G, Grossman A
Endocr Relat Cancer 2016 Sep;23(9):R423-36. Epub 2016 Jul 26 doi: 10.1530/ERC-16-0200. PMID: 27461388
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
Management of gastroentero-pancreatic neuroendocrine tumors (GEP-NETs).
Desai KK, Khan MS, Toumpanakis C, Caplin ME
Minerva Gastroenterol Dietol 2009 Dec;55(4):425-43. PMID: 19942827
VIPoma syndrome.
Mekhjian HS, O'Dorisio TM
Semin Oncol 1987 Sep;14(3):282-91. PMID: 2820063

Prognosis

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: 37905620Free 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
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
Anderson CW, Bennett JJ
Surg Oncol Clin N Am 2016 Apr;25(2):363-74. Epub 2016 Feb 18 doi: 10.1016/j.soc.2015.12.003. PMID: 27013370
Pancreatic neuroendocrine tumours.
Ruiz-Tovar J, Priego P, Martínez-Molina E, Morales V, Sanjuanbenito A, Lobo E
Clin Transl Oncol 2008 Aug;10(8):493-7. doi: 10.1007/s12094-008-0238-1. PMID: 18667380
Multiple endocrine neoplasia syndromes.
Lips CJ, Vasen HF, Lamers CB
Crit Rev Oncol Hematol 1984;2(2):117-84. doi: 10.1016/s1040-8428(84)80017-7. PMID: 6152202

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: 27501174Free 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

Recent systematic reviews

Clinicopathological data and treatment modalities for pancreatic vipomas: a systematic review.
Schizas D, Mastoraki A, Bagias G, Patras R, Moris D, Lazaridis II, Arkadopoulos N, Felekouras E
J BUON 2019 Mar-Apr;24(2):415-423. PMID: 31127985

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