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Somatostatinoma

MedGen UID:
52416
Concept ID:
C0037661
Neoplastic Process
Synonyms: Ampullary somatostatinoma; Carcinoid somatostatinoma; Malignant islet cell tumor; Somatomedin-secreting carcinoid; Somatostatin-secreting pancreatic neoplasm
SNOMED CT: Somatostatin cell tumor (128642005); Somatostatinoma (128642005); Delta cell tumor (253006001); Somatostatinoma (253006001)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0006976
Orphanet: ORPHA97283

Definition

Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor (see these terms) that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria. [from ORDO]

Professional guidelines

PubMed

Popoviciu MS, Paduraru L, Nutas RM, Ujoc AM, Yahya G, Metwally K, Cavalu S
Int J Mol Sci 2023 Aug 11;24(16) doi: 10.3390/ijms241612676. PMID: 37628857Free PMC Article
Mastoraki A, Schizas D, Papoutsi E, Ntella V, Kanavidis P, Sioulas A, Tsoli M, Charalampopoulos G, Vailas M, Felekouras E
In Vivo 2020 Nov-Dec;34(6):3573-3582. doi: 10.21873/invivo.12201. PMID: 33144470Free PMC Article
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

Recent clinical studies

Etiology

Popoviciu MS, Paduraru L, Nutas RM, Ujoc AM, Yahya G, Metwally K, Cavalu S
Int J Mol Sci 2023 Aug 11;24(16) doi: 10.3390/ijms241612676. PMID: 37628857Free PMC Article
Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
Därr R, Nambuba J, Del Rivero J, Janssen I, Merino M, Todorovic M, Balint B, Jochmanova I, Prchal JT, Lechan RM, Tischler AS, Popovic V, Miljic D, Adams KT, Prall FR, Ling A, Golomb MR, Ferguson M, Nilubol N, Chen CC, Chew E, Taïeb D, Stratakis CA, Fojo T, Yang C, Kebebew E, Zhuang Z, Pacak K
Endocr Relat Cancer 2016 Dec;23(12):899-908. Epub 2016 Sep 27 doi: 10.1530/ERC-16-0231. PMID: 27679736Free PMC Article
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
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

Alzahrani AS, Alswailem M, Buffet A, Alghamdi B, Alobaid L, Alsagheir O, Al-Hindi H, Pacak K
Endocr Relat Cancer 2024 Aug 1;31(8) Epub 2024 Jun 10 doi: 10.1530/ERC-23-0303. PMID: 38767322
Popoviciu MS, Paduraru L, Nutas RM, Ujoc AM, Yahya G, Metwally K, Cavalu S
Int J Mol Sci 2023 Aug 11;24(16) doi: 10.3390/ijms241612676. PMID: 37628857Free PMC Article
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
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
Soldati T, Del Noce G, Garino M, Farina EC, De Paolis P, Balbo G
Panminerva Med 1990 Jul-Sep;32(3):141-4. PMID: 2077482

Therapy

Yu R, Rao J, Zhai J, Hecht JR, Pisegna JR
Pancreas 2022 Sep 1;51(8):1056-1060. doi: 10.1097/MPA.0000000000002126. PMID: 36607953
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
Resmini E, Minuto F, Colao A, Ferone D
Acta Diabetol 2009 Jun;46(2):85-95. Epub 2009 Mar 26 doi: 10.1007/s00592-009-0112-9. PMID: 19322513
Doherty GM
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):807-17. doi: 10.1016/j.bpg.2005.05.004. PMID: 16253902
Lamers CB
Acta Endocrinol Suppl (Copenh) 1987;286:19-25. doi: 10.1530/acta.0.115s019. PMID: 2892334

Prognosis

Lam AK, Ishida H
Histol Histopathol 2021 Apr;36(4):367-382. Epub 2020 Dec 11 doi: 10.14670/HH-18-288. PMID: 33305819
Jochmanová I, Zelinka T, Widimský J Jr, Pacak K
Physiol Res 2014;63(Suppl 2):S251-62. doi: 10.33549/physiolres.932789. PMID: 24908231
Di Florio A, Sancho V, Moreno P, Delle Fave G, Jensen RT
Biochim Biophys Acta 2013 Mar;1833(3):573-82. Epub 2012 Dec 4 doi: 10.1016/j.bbamcr.2012.11.021. PMID: 23220008Free PMC Article
Morgan KA, Adams DB
Surg Clin North Am 2010 Apr;90(2):287-307. doi: 10.1016/j.suc.2009.12.009. PMID: 20362787
Soldati T, Del Noce G, Garino M, Farina EC, De Paolis P, Balbo G
Panminerva Med 1990 Jul-Sep;32(3):141-4. PMID: 2077482

Clinical prediction guides

Dwight T, Kim E, Bastard K, Benn DE, Eisenhofer G, Richter S, Mannelli M, Rapizzi E, Prejbisz A, Pęczkowska M, Pacak K, Clifton-Bligh R
Endocr Relat Cancer 2021 Feb;28(2):97-109. doi: 10.1530/ERC-20-0280. PMID: 33300499Free PMC Article
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
Usui M, Matsuda S, Suzuki H, Hirata K, Ogura Y, Shiraishi T
J Gastroenterol 2002;37(11):947-53. doi: 10.1007/s005350200159. PMID: 12483251
Azimuddin K, Chamberlain RS
Surg Clin North Am 2001 Jun;81(3):511-25. doi: 10.1016/s0039-6109(05)70140-7. PMID: 11459268
Howard TJ, Stabile BE, Zinner MJ, Chang S, Bhagavan BS, Passaro E Jr
Am J Surg 1990 Feb;159(2):258-64. doi: 10.1016/s0002-9610(05)80276-4. PMID: 2154144

Recent systematic reviews

Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044

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