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Familial pancreatic carcinoma

MedGen UID:
419700
•Concept ID:
C2931038
•
Neoplastic Process
Synonym: Familial Pancreatic Cancer
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
Autosomal dominant inheritance (Orphanet)
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0015278
Orphanet: ORPHA1333

Definition

Familial pancreatic carcinoma is defined by the presence of pancreatic cancer (PC) in two or more first-degree relatives. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Familial pancreatic carcinoma in Orphanet.

Professional guidelines

PubMed

Molecular Features and Clinical Management of Hereditary Pancreatic Cancer Syndromes and Familial Pancreatic Cancer.
Kasuga A, Okamoto T, Udagawa S, Mori C, Mie T, Furukawa T, Yamada Y, Takeda T, Matsuyama M, Sasaki T, Ozaka M, Ueki A, Sasahira N
Int J Mol Sci 2022 Jan 21;23(3) doi: 10.3390/ijms23031205. PMID: 35163129Free PMC Article
Management of patients with increased risk for familial pancreatic cancer: updated recommendations from the International Cancer of the Pancreas Screening (CAPS) Consortium.
Goggins M, Overbeek KA, Brand R, Syngal S, Del Chiaro M, Bartsch DK, Bassi C, Carrato A, Farrell J, Fishman EK, Fockens P, Gress TM, van Hooft JE, Hruban RH, Kastrinos F, Klein A, Lennon AM, Lucas A, Park W, Rustgi A, Simeone D, Stoffel E, Vasen HFA, Cahen DL, Canto MI, Bruno M; International Cancer of the Pancreas Screening (CAPS) consortium
Gut 2020 Jan;69(1):7-17. Epub 2019 Oct 31 doi: 10.1136/gutjnl-2019-319352. PMID: 31672839Free PMC Article
Familial pancreatic cancer: Concept, management and issues.
Matsubayashi H, Takaori K, Morizane C, Maguchi H, Mizuma M, Takahashi H, Wada K, Hosoi H, Yachida S, Suzuki M, Usui R, Furukawa T, Furuse J, Sato T, Ueno M, Kiyozumi Y, Hijioka S, Mizuno N, Terashima T, Mizumoto M, Kodama Y, Torishima M, Kawaguchi T, Ashida R, Kitano M, Hanada K, Furukawa M, Kawabe K, Majima Y, Shimosegawa T
World J Gastroenterol 2017 Feb 14;23(6):935-948. doi: 10.3748/wjg.v23.i6.935. PMID: 28246467Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology, Genetic/Familial High-Risk Assessment: Breast, Ovarian, and Pancreatic version 2.2022

Recent clinical studies

Etiology

Surveillance of high-risk individuals for pancreatic cancer with EUS and MRI: A meta-analysis.
Kogekar N, Diaz KE, Weinberg AD, Lucas AL
Pancreatology 2020 Dec;20(8):1739-1746. Epub 2020 Oct 9 doi: 10.1016/j.pan.2020.10.025. PMID: 33077384
Risk Factors in Pancreatic Adenocarcinoma: the Interrelation with Familial History and Predictive Role on Survival.
Petrusel L, Bilibou M, Drug V, Leucuta DC, Seicean R, Cainap C, Seicean A
J Gastrointestin Liver Dis 2020 Sep 9;29(3):391-398. doi: 10.15403/jgld-2529. PMID: 32919422
Risk factors associated with earlier age of onset in familial pancreatic carcinoma.
James TA, Sheldon DG, Rajput A, Kuvshinoff BW, Javle MM, Nava HR, Smith JL, Gibbs JF
Cancer 2004 Dec 15;101(12):2722-6. doi: 10.1002/cncr.20700. PMID: 15534880
Familial pancreatic carcinoma in Jews.
Lynch HT, Deters CA, Lynch JF, Brand RE
Fam Cancer 2004;3(3-4):233-40. doi: 10.1007/s10689-004-9549-8. PMID: 15516847
BRCA2 germline mutations in familial pancreatic carcinoma.
Hahn SA, Greenhalf B, Ellis I, Sina-Frey M, Rieder H, Korte B, Gerdes B, Kress R, Ziegler A, Raeburn JA, Campra D, Grützmann R, Rehder H, Rothmund M, Schmiegel W, Neoptolemos JP, Bartsch DK
J Natl Cancer Inst 2003 Feb 5;95(3):214-21. doi: 10.1093/jnci/95.3.214. PMID: 12569143

Diagnosis

Risk factors associated with earlier age of onset in familial pancreatic carcinoma.
James TA, Sheldon DG, Rajput A, Kuvshinoff BW, Javle MM, Nava HR, Smith JL, Gibbs JF
Cancer 2004 Dec 15;101(12):2722-6. doi: 10.1002/cncr.20700. PMID: 15534880
Familial pancreatic carcinoma in Jews.
Lynch HT, Deters CA, Lynch JF, Brand RE
Fam Cancer 2004;3(3-4):233-40. doi: 10.1007/s10689-004-9549-8. PMID: 15516847
Characterization of FAMPAC, a newly identified human pancreatic carcinoma cell line with a hereditary background.
Eisold S, Ryschich E, Linnebacher M, Giese T, Nauheimer D, Wild A, Bartsch DK, Büchler MW, Schmidt J
Cancer 2004 May 1;100(9):1978-86. doi: 10.1002/cncr.20193. PMID: 15112280
BRCA2 germline mutations in familial pancreatic carcinoma.
Hahn SA, Greenhalf B, Ellis I, Sina-Frey M, Rieder H, Korte B, Gerdes B, Kress R, Ziegler A, Raeburn JA, Campra D, Grützmann R, Rehder H, Rothmund M, Schmiegel W, Neoptolemos JP, Bartsch DK
J Natl Cancer Inst 2003 Feb 5;95(3):214-21. doi: 10.1093/jnci/95.3.214. PMID: 12569143
Increased risk of incident pancreatic cancer among first-degree relatives of patients with familial pancreatic cancer.
Tersmette AC, Petersen GM, Offerhaus GJ, Falatko FC, Brune KA, Goggins M, Rozenblum E, Wilentz RE, Yeo CJ, Cameron JL, Kern SE, Hruban RH
Clin Cancer Res 2001 Mar;7(3):738-44. PMID: 11297271

Therapy

Surveillance of high-risk individuals for pancreatic cancer with EUS and MRI: A meta-analysis.
Kogekar N, Diaz KE, Weinberg AD, Lucas AL
Pancreatology 2020 Dec;20(8):1739-1746. Epub 2020 Oct 9 doi: 10.1016/j.pan.2020.10.025. PMID: 33077384
Risk Factors in Pancreatic Adenocarcinoma: the Interrelation with Familial History and Predictive Role on Survival.
Petrusel L, Bilibou M, Drug V, Leucuta DC, Seicean R, Cainap C, Seicean A
J Gastrointestin Liver Dis 2020 Sep 9;29(3):391-398. doi: 10.15403/jgld-2529. PMID: 32919422

Prognosis

Risk Factors in Pancreatic Adenocarcinoma: the Interrelation with Familial History and Predictive Role on Survival.
Petrusel L, Bilibou M, Drug V, Leucuta DC, Seicean R, Cainap C, Seicean A
J Gastrointestin Liver Dis 2020 Sep 9;29(3):391-398. doi: 10.15403/jgld-2529. PMID: 32919422
Appropriateness of pancreatic resection in high-risk individuals for familial pancreatic ductal adenocarcinoma: a patient-level meta-analysis and proposition of the Beaujon score.
de Mestier L, Muller M, Cros J, Vullierme MP, Vernerey D, Maire F, Dokmak S, Rebours V, Sauvanet A, Lévy P, Hammel P
United European Gastroenterol J 2019 Apr;7(3):358-368. Epub 2019 Jan 12 doi: 10.1177/2050640618824910. PMID: 31019704Free PMC Article
Familial pancreatic carcinoma in Jews.
Lynch HT, Deters CA, Lynch JF, Brand RE
Fam Cancer 2004;3(3-4):233-40. doi: 10.1007/s10689-004-9549-8. PMID: 15516847
Characterization of FAMPAC, a newly identified human pancreatic carcinoma cell line with a hereditary background.
Eisold S, Ryschich E, Linnebacher M, Giese T, Nauheimer D, Wild A, Bartsch DK, Büchler MW, Schmidt J
Cancer 2004 May 1;100(9):1978-86. doi: 10.1002/cncr.20193. PMID: 15112280
BRCA2 germline mutations in familial pancreatic carcinoma.
Hahn SA, Greenhalf B, Ellis I, Sina-Frey M, Rieder H, Korte B, Gerdes B, Kress R, Ziegler A, Raeburn JA, Campra D, Grützmann R, Rehder H, Rothmund M, Schmiegel W, Neoptolemos JP, Bartsch DK
J Natl Cancer Inst 2003 Feb 5;95(3):214-21. doi: 10.1093/jnci/95.3.214. PMID: 12569143

Clinical prediction guides

Risk Factors in Pancreatic Adenocarcinoma: the Interrelation with Familial History and Predictive Role on Survival.
Petrusel L, Bilibou M, Drug V, Leucuta DC, Seicean R, Cainap C, Seicean A
J Gastrointestin Liver Dis 2020 Sep 9;29(3):391-398. doi: 10.15403/jgld-2529. PMID: 32919422
Appropriateness of pancreatic resection in high-risk individuals for familial pancreatic ductal adenocarcinoma: a patient-level meta-analysis and proposition of the Beaujon score.
de Mestier L, Muller M, Cros J, Vullierme MP, Vernerey D, Maire F, Dokmak S, Rebours V, Sauvanet A, Lévy P, Hammel P
United European Gastroenterol J 2019 Apr;7(3):358-368. Epub 2019 Jan 12 doi: 10.1177/2050640618824910. PMID: 31019704Free PMC Article
Risk factors associated with earlier age of onset in familial pancreatic carcinoma.
James TA, Sheldon DG, Rajput A, Kuvshinoff BW, Javle MM, Nava HR, Smith JL, Gibbs JF
Cancer 2004 Dec 15;101(12):2722-6. doi: 10.1002/cncr.20700. PMID: 15534880
BRCA2 germline mutations in familial pancreatic carcinoma.
Hahn SA, Greenhalf B, Ellis I, Sina-Frey M, Rieder H, Korte B, Gerdes B, Kress R, Ziegler A, Raeburn JA, Campra D, Grützmann R, Rehder H, Rothmund M, Schmiegel W, Neoptolemos JP, Bartsch DK
J Natl Cancer Inst 2003 Feb 5;95(3):214-21. doi: 10.1093/jnci/95.3.214. PMID: 12569143
Familial head and neck cancer: molecular analysis of a new clinical entity.
Yu KK, Zanation AM, Moss JR, Yarbrough WG
Laryngoscope 2002 Sep;112(9):1587-93. doi: 10.1097/00005537-200209000-00010. PMID: 12352668

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2022
      NCCN Clinical Practice Guidelines in Oncology, Genetic/Familial High-Risk Assessment: Breast, Ovarian, and Pancreatic version 2.2022

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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