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Non-functional pancreatic neuroendocrine tumor

MedGen UID:
277416
Concept ID:
C1334977
Neoplastic Process
Synonyms: Non functioning pancreatic endocrine tumor; Non-functioning endocrine pancreatic tumors
SNOMED CT: Pancreatic endocrine tumor, nonfunctioning (703816006); Non-functioning PNET (pancreatic neuroendocrine tumor) (1208744003); Non-functioning well-differentiated pancreatic NEN (neuroendocrine neoplasm) (1208744003); Non-functioning neuroendocrine neoplasm of pancreas (1208744003); Non-functioning well-differentiated neuroendocrine neoplasm of pancreas (1208744003); Pancreatic neuroendocrine tumor, nonfunctioning (703816006); Pancreatic neuroendocrine neoplasm, non-functioning (703816006)
 
Monarch Initiative: MONDO:0004334
Orphanet: ORPHA506075

Definition

A rare neuroendocrine tumor of pancreas characterized by a well-differentiated epithelial pancreatic neuroendocrine neoplasm measuring at least 0.5 cm, without distinct hormonal syndrome. Tumors less than 0.5 cm are called microadenomas. Microadenomatosis is the multifocal occurrence of microadenomas. Histopathologic examination shows an organoid growth pattern and expression of synaptophysin and chromogranin A on immunohistochemistry. Tumors are often discovered incidentally, or patients may present with symptoms related to local or metastatic tumor spread. Microadenomas are considered benign, while larger tumors may behave in a malignant manner with extrapancreatic spread, metastasis, or recurrence. [from SNOMEDCT_US]

Professional guidelines

PubMed

Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Recent clinical studies

Etiology

Marx M, Godat S, Caillol F, Poizat F, Ratone JP, Pesenti C, Schoepfer A, Hoibian S, Dahel Y, Giovannini M
Dig Endosc 2022 Sep;34(6):1207-1213. Epub 2022 Jan 19 doi: 10.1111/den.14224. PMID: 34963025
Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127
Mirică A, Bădărău IA, Mirică R, Păun S, Păun DL
J Med Life 2016 Oct-Dec;9(4):369-372. PMID: 27928440Free PMC Article
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Diagnosis

He M, Liu Z, Lin Y, Wan J, Li J, Xu K, Wang Y, Jin Z, Tian J, Xue H
Eur J Radiol 2019 Aug;117:102-111. Epub 2019 May 31 doi: 10.1016/j.ejrad.2019.05.024. PMID: 31307634
Varedi D, Frigerio A, Scaife C, Hull C
Dermatol Online J 2019 Mar 15;25(3) PMID: 30982303
Mirică A, Bădărău IA, Mirică R, Păun S, Păun DL
J Med Life 2016 Oct-Dec;9(4):369-372. PMID: 27928440Free PMC Article
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Rustagi T, Rai M, Bauer F
J Gastrointest Cancer 2013 Sep;44(3):336-42. doi: 10.1007/s12029-012-9445-7. PMID: 23065708

Therapy

Mirică A, Bădărău IA, Mirică R, Păun S, Păun DL
J Med Life 2016 Oct-Dec;9(4):369-372. PMID: 27928440Free PMC Article
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Prognosis

Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Rustagi T, Rai M, Bauer F
J Gastrointest Cancer 2013 Sep;44(3):336-42. doi: 10.1007/s12029-012-9445-7. PMID: 23065708

Clinical prediction guides

Marx M, Godat S, Caillol F, Poizat F, Ratone JP, Pesenti C, Schoepfer A, Hoibian S, Dahel Y, Giovannini M
Dig Endosc 2022 Sep;34(6):1207-1213. Epub 2022 Jan 19 doi: 10.1111/den.14224. PMID: 34963025
Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127

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