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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: 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
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
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

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
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
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
Hoffmann KM, Furukawa M, Jensen RT
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):675-97. doi: 10.1016/j.bpg.2005.05.009. PMID: 16253893

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
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
Mansour JC, Chen H
J Surg Res 2004 Jul;120(1):139-61. doi: 10.1016/j.jss.2003.12.007. PMID: 15172200

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
Wright J, Abolfathi A, Penman E, Marks V
Clin Endocrinol (Oxf) 1980 Jun;12(6):603-8. doi: 10.1111/j.1365-2265.1980.tb01382.x. PMID: 6105026

Prognosis

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
Ellison TA, Edil BH
Adv Surg 2012;46:283-96. doi: 10.1016/j.yasu.2012.04.002. PMID: 22873046
Cheng SP, Doherty GM
Cancer Treat Res 2010;153:253-70. doi: 10.1007/978-1-4419-0857-5_14. PMID: 19957229
Hoffmann KM, Furukawa M, Jensen RT
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):675-97. doi: 10.1016/j.bpg.2005.05.009. PMID: 16253893
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

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
Killingsworth MC, Bobryshev YV
J Vis Exp 2016 Aug 7;(114) doi: 10.3791/54307. PMID: 27584907Free PMC Article
Brentjens R, Saltz L
Surg Clin North Am 2001 Jun;81(3):527-42. doi: 10.1016/s0039-6109(05)70141-9. PMID: 11459269
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
Stamm B, Hedinger CE, Saremaslani P
Virchows Arch A Pathol Anat Histopathol 1986;408(5):475-89. doi: 10.1007/BF00705301. PMID: 2869609

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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