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Pancreatic acinar cell carcinoma

MedGen UID:
83540
Concept ID:
C0279661
Neoplastic Process
Synonyms: Acinar Cell Adenocarcinoma of Pancreas; acinar cell adenocarcinoma of pancreas; Acinar Cell Adenocarcinoma of the Pancreas; acinar cell adenocarcinoma of the pancreas; Acinar cell carcinoma of pancreas; Acinar Cell Carcinoma of Pancreas; acinar cell carcinoma of pancreas; Acinar Cell Carcinoma of the Pancreas; acinar cell carcinoma of the pancreas; carcinoma of pancreatic acinar cell; PAAC; Pancreas Acinar Cell Adenocarcinoma; pancreas acinar cell adenocarcinoma; Pancreatic Acinar Cell Adenocarcinoma; pancreatic acinar cell adenocarcinoma; Pancreatic acinar cell cancer; Pancreatic Acinar Cell Carcinoma; pancreatic acinar cell carcinoma
SNOMED CT: Pancreatic acinar cell carcinoma (783771003); Acinar cell carcinoma of pancreas (783771003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0006346
Orphanet: ORPHA424046

Definition

A very rare, malignant, epithelial tumor of the pancreas characterized, macroscopically, by a usually large, well-circumscribed, fully or partially encapsulated, solid mass, often with hemorrhage, necrosis and cystic changes, in any portion of the pancreas and, histologically, by neoplastic cells with variable degrees of differentiation and morphology, ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome, familial adenomatous polyposis, and pancreatic panniculitis has been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic acinar cell carcinoma

Professional guidelines

PubMed

Ikezawa K, Urabe M, Kai Y, Takada R, Akita H, Nagata S, Ohkawa K
Jpn J Clin Oncol 2024 Mar 9;54(3):271-281. doi: 10.1093/jjco/hyad176. PMID: 38109477Free PMC Article
Garajová I, Peroni M, Gelsomino F, Leonardi F
Curr Oncol 2023 Oct 31;30(11):9587-9601. doi: 10.3390/curroncol30110694. PMID: 37999114Free PMC Article
Oh YL, Song SY, Ahn G
Appl Immunohistochem Mol Morphol 2002 Mar;10(1):62-6. doi: 10.1097/00129039-200203000-00011. PMID: 11893038

Recent clinical studies

Etiology

Garajová I, Peroni M, Gelsomino F, Leonardi F
Curr Oncol 2023 Oct 31;30(11):9587-9601. doi: 10.3390/curroncol30110694. PMID: 37999114Free PMC Article
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Jornet D, Soyer P, Terris B, Hoeffel C, Oudjit A, Legmann P, Gaujoux S, Barret M, Dohan A
Diagn Interv Imaging 2019 Jul-Aug;100(7-8):427-435. Epub 2019 Mar 4 doi: 10.1016/j.diii.2019.02.003. PMID: 30846400
Luo Y, Hu G, Ma Y, Guo N, Li F
Medicine (Baltimore) 2017 Sep;96(38):e7904. doi: 10.1097/MD.0000000000007904. PMID: 28930825Free PMC Article
Hu S, Hu S, Wang M, Wu Z, Miao F
Radiol Med 2013 Aug;118(5):723-31. Epub 2013 Jan 28 doi: 10.1007/s11547-012-0908-5. PMID: 23358813

Diagnosis

Mandelker D, Marra A, Zheng-Lin B, Selenica P, Blanco-Heredia J, Zhu Y, Gazzo A, Wong D, Yelskaya Z, Rai V, Somar J, Ostafi S, Mehta N, Yang C, Li Y, Brown DN, da Silva EM, Pei X, Linkov I, Terraf P, Misyura M, Ceyhan-Birsoy O, Ladanyi M, Berger M, Pareja F, Stadler Z, Offit K, Riaz N, Park W, Chou J, Capanu M, Koehler M, Rosen E, O'Reilly EM, Reis-Filho JS
J Clin Oncol 2023 Nov 20;41(33):5151-5162. Epub 2023 Aug 22 doi: 10.1200/JCO.23.00561. PMID: 37607324Free PMC Article
Sun T, Gilani S, Jain D, Cai G
Diagn Cytopathol 2023 Nov;51(11):674-683. Epub 2023 Jul 19 doi: 10.1002/dc.25196. PMID: 37469257
Kryklyva V, Haj Mohammad N, Morsink FHM, Ligtenberg MJL, Offerhaus GJA, Nagtegaal ID, de Leng WWJ, Brosens LAA
Cancer Biol Ther 2019;20(7):949-955. Epub 2019 Apr 19 doi: 10.1080/15384047.2019.1595274. PMID: 31002019Free PMC Article
Gandhi NS, Feldman MK, Le O, Morris-Stiff G
Abdom Radiol (NY) 2018 Feb;43(2):273-284. doi: 10.1007/s00261-017-1330-1. PMID: 29038855
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article

Therapy

Garajová I, Peroni M, Gelsomino F, Leonardi F
Curr Oncol 2023 Oct 31;30(11):9587-9601. doi: 10.3390/curroncol30110694. PMID: 37999114Free PMC Article
Lee CL, Holter S, Borgida A, Dodd A, Ramotar S, Grant R, Wasson K, Elimova E, Jang RW, Moore M, Kim TK, Khalili K, Moulton CA, Gallinger S, O'Kane GM, Knox JJ
World J Gastroenterol 2022 Dec 7;28(45):6421-6432. doi: 10.3748/wjg.v28.i45.6421. PMID: 36533108Free PMC Article
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article
Seo S, Yoo C, Kim KP, Ryoo BY, Chang HM, Hong SM, Lee JH, Song KB, Hwang DW, Kim KH, Hwang S, Kim SC
J Dig Dis 2017 Aug;18(8):480-486. doi: 10.1111/1751-2980.12505. PMID: 28671770
Matos JM, Schmidt CM, Turrini O, Agaram NP, Niedergethmann M, Saeger HD, Merchant N, Johnson CS, Lillemoe KD, Grützmann R
J Gastrointest Surg 2009 Aug;13(8):1495-502. Epub 2009 Jun 3 doi: 10.1007/s11605-009-0938-z. PMID: 19495891

Prognosis

Ikezawa K, Urabe M, Kai Y, Takada R, Akita H, Nagata S, Ohkawa K
Jpn J Clin Oncol 2024 Mar 9;54(3):271-281. doi: 10.1093/jjco/hyad176. PMID: 38109477Free PMC Article
Garajová I, Peroni M, Gelsomino F, Leonardi F
Curr Oncol 2023 Oct 31;30(11):9587-9601. doi: 10.3390/curroncol30110694. PMID: 37999114Free PMC Article
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Kryklyva V, Haj Mohammad N, Morsink FHM, Ligtenberg MJL, Offerhaus GJA, Nagtegaal ID, de Leng WWJ, Brosens LAA
Cancer Biol Ther 2019;20(7):949-955. Epub 2019 Apr 19 doi: 10.1080/15384047.2019.1595274. PMID: 31002019Free PMC Article
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article

Clinical prediction guides

Sun T, Gilani S, Jain D, Cai G
Diagn Cytopathol 2023 Nov;51(11):674-683. Epub 2023 Jul 19 doi: 10.1002/dc.25196. PMID: 37469257
Uruga H, Tamura T, Miura Y, Itonaga J, Koyama R, Imamura T, Takazawa Y
Pathol Int 2023 Jun;73(6):255-260. Epub 2023 May 3 doi: 10.1111/pin.13326. PMID: 37133201
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Xing-Mao Z, Hong-Juan Z, Qing L, Qiang H
BMC Cancer 2018 Nov 8;18(1):1083. doi: 10.1186/s12885-018-5008-z. PMID: 30409114Free PMC Article
He R, Yin Y, Yin W, Li Y, Zhao J, Zhang W
Nat Commun 2018 Oct 10;9(1):4183. doi: 10.1038/s41467-018-06571-w. PMID: 30305629Free PMC Article

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