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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
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
de Herder WW, Rehfeld JF, Kidd M, Modlin IM
Best Pract Res Clin Endocrinol Metab 2016 Jan;30(1):3-17. Epub 2015 Oct 23 doi: 10.1016/j.beem.2015.10.004. PMID: 26971840
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
Oberg K, Eriksson B
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):753-81. doi: 10.1016/j.bpg.2005.06.002. PMID: 16253899

Diagnosis

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
Vinik AI, Strodel WE, Eckhauser FE, Moattari AR, Lloyd R
Semin Oncol 1987 Sep;14(3):263-81. PMID: 2820062

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
Doherty GM
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):807-17. doi: 10.1016/j.bpg.2005.05.004. PMID: 16253902
Virgolini I, Traub-Weidinger T, Decristoforo C
Best Pract Res Clin Endocrinol Metab 2005 Jun;19(2):213-27. doi: 10.1016/j.beem.2004.09.001. PMID: 15763696
Lamers CB
Acta Endocrinol Suppl (Copenh) 1987;286:19-25. doi: 10.1530/acta.0.115s019. PMID: 2892334

Prognosis

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
Morgan KA, Adams DB
Surg Clin North Am 2010 Apr;90(2):287-307. doi: 10.1016/j.suc.2009.12.009. PMID: 20362787
Oberg K, Eriksson B
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):753-81. doi: 10.1016/j.bpg.2005.06.002. PMID: 16253899
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
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

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
Bomanji JB, Wong W, Gaze MN, Cassoni A, Waddington W, Solano J, Ell PJ
Clin Oncol (R Coll Radiol) 2003 Jun;15(4):193-8. doi: 10.1016/s0936-6555(02)00273-x. PMID: 12846498
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
Shen LP, Pictet RL, Rutter WJ
Proc Natl Acad Sci U S A 1982 Aug;79(15):4575-9. doi: 10.1073/pnas.79.15.4575. PMID: 6126875Free PMC Article

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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