Familial Pancreatic Cancer Syndromes

2006 ◽  
Vol 35 (2) ◽  
pp. 417-430 ◽  
Author(s):  
Nils Habbe ◽  
Peter Langer ◽  
Mercedes Sina-Frey ◽  
Detlef K. Bartsch
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cancer Syndromes

scholarly journals Surveillance of Individuals with a Family History of Pancreatic Cancer and Inherited Cancer Syndromes: A Strategy for Detecting Early Pancreatic Cancers

Diagnostics ◽  
2019 ◽  
Vol 9 (4) ◽  
pp. 169
Author(s):  
Matsubayashi ◽  
Kiyozumi ◽  
Ishiwatari ◽  
Uesaka ◽  
Kikuyama ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Family History ◽  
Early Stage ◽  
Developed Countries ◽  
Familial Pancreatic Cancer ◽  
Cancer Syndrome ◽  
Inherited Cancer ◽  
Clinicopathological Findings ◽  
History Of ◽  
Cancer Syndromes

A family history of pancreatic cancer (PC) is a risk factor of PC, and risk levels increase as affected families grow in number and/or develop PC at younger ages. Familial pancreatic cancer (FPC) is defined as a client having at least two PC cases in a first degree relatives. In the narrow sense, FPC does not include some inherited cancer syndromes that are known to increase the risks of PC, such as Peutz–Jeghers syndrome (PJS), hereditary pancreatitis (HP), hereditary breast ovarian cancer syndrome (HBOC), and so on. FPC accounts for 5%–10% of total PC diagnoses and is marked by several features in genetic, epidemiological, and clinicopathological findings that are similar to or distinct from conventional PC. Recent advances in genetic medicine have led to an increased ability to identify germline variants of cancer-associated genes. To date, high-risk individuals (HRIs) in many developed countries, including FPC kindreds and inherited cancer syndromes, are screened clinically to detect and treat early-stage PC. This article highlights the concept of FPC and the most recent data on its detection.

scholarly journals Genomic Features and Clinical Management of Patients with Hereditary Pancreatic Cancer Syndromes and Familial Pancreatic Cancer

2019 ◽  
Vol 20 (3) ◽  
pp. 561 ◽  
Author(s):  
Akihiro Ohmoto ◽  
Shinichi Yachida ◽  
Chigusa Morizane
Keyword(s):  
Pancreatic Cancer ◽  
Checkpoint Inhibitors ◽  
Susceptibility Gene ◽  
Treatment Strategies ◽  
Familial Pancreatic Cancer ◽  
Cancer Syndrome ◽  
Increased Risk ◽  
Clinical Benefits ◽  
Surveillance Programs ◽  
Cancer Syndromes

Pancreatic cancer (PC) is one of the most devastating malignancies; it has a 5-year survival rate of only 9%, and novel treatment strategies are urgently needed. While most PC cases occur sporadically, PC associated with hereditary syndromes or familial PC (FPC; defined as an individual having two or more first-degree relatives diagnosed with PC) accounts for about 10% of cases. Hereditary cancer syndromes associated with increased risk for PC include Peutz-Jeghers syndrome, hereditary pancreatitis, familial atypical multiple mole melanoma, familial adenomatous polyposis, Lynch syndrome and hereditary breast and ovarian cancer syndrome. Next-generation sequencing of FPC patients has uncovered new susceptibility genes such as PALB2 and ATM, which participate in homologous recombination repair, and further investigations are in progress. Previous studies have demonstrated that some sporadic cases that do not fulfil FPC criteria also harbor similar mutations, and so genomic testing based on family history might overlook some susceptibility gene carriers. There are no established screening procedures for high-risk unaffected cases, and it is not clear whether surveillance programs would have clinical benefits. In terms of treatment, poly (ADP-ribose) polymerase inhibitors for BRCA-mutated cases or immune checkpoint inhibitors for mismatch repair deficient cases are promising, and clinical trials of these agents are underway.

scholarly journals Characteristics of early‐onset pancreatic cancer and its association with familial pancreatic cancer and hereditary pancreatic cancer syndromes

2020 ◽  
Vol 4 (3) ◽  
pp. 229-233 ◽  
Author(s):  
Hidetoshi Eguchi ◽  
Shogo Kobayashi ◽  
Kunihito Gotoh ◽  
Takehiro Noda ◽  
Yuichiro Doki
Keyword(s):  
Pancreatic Cancer ◽  
Early Onset ◽  
Familial Pancreatic Cancer ◽  
Hereditary Pancreatic Cancer ◽  
Cancer Syndromes

scholarly journals Somatic Mutation Profiling in the Liquid Biopsy and Clinical Analysis of Hereditary and Familial Pancreatic Cancer Cases Reveals KRAS Negativity and a Longer Overall Survival

Cancers ◽  
2021 ◽  
Vol 13 (7) ◽  
pp. 1612
Author(s):  
Julie Earl ◽  
Emma Barreto ◽  
María E. Castillo ◽  
Raquel Fuentes ◽  
Mercedes Rodríguez-Garrote ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Overall Survival ◽  
Somatic Mutation ◽  
Disease Status ◽  
Clinical Analysis ◽  
Cytotoxic Agents ◽  
Disease Stage ◽  
Ductal Adenocarcinoma ◽  
Familial Pancreatic Cancer ◽  
Circulating Free Dna

Pancreatic ductal adenocarcinoma (PDAC) presents many challenges in the clinic and there are many areas for improvement in diagnostics and patient management. The five-year survival rate is around 7.2% as the majority of patients present with advanced disease at diagnosis that is treatment resistant. Approximately 10–15% of PDAC cases have a hereditary basis or Familial Pancreatic Cancer (FPC). Here we demonstrate the use of circulating free DNA (cfDNA) in plasma as a prognostic biomarker in PDAC. The levels of cfDNA correlated with disease status, disease stage, and overall survival. Furthermore, we show for the first time via BEAMing that the majority of hereditary or familial PDAC cases (around 84%) are negative for a KRAS somatic mutation. In addition, KRAS mutation negative cases harbor somatic mutations in potentially druggable genes such as KIT, PDGFR, MET, BRAF, and PIK3CA that could be exploited in the clinic. Finally, familial or hereditary cases have a longer overall survival compared to sporadic cases (10.2 vs. 21.7 months, respectively). Currently, all patients are treated the same in the clinic with cytotoxic agents, although here we demonstrate that there are different subtypes of tumors at the genetic level that could pave the way to personalized treatment.

scholarly journals Whole Genome Sequencing Defines the Genetic Heterogeneity of Familial Pancreatic Cancer

2015 ◽  
Vol 6 (2) ◽  
pp. 166-175 ◽  
Author(s):  
Nicholas J. Roberts ◽  
Alexis L. Norris ◽  
Gloria M. Petersen ◽  
Melissa L. Bondy ◽  
Randall Brand ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Whole Genome Sequencing ◽  
Genome Sequencing ◽  
Genetic Heterogeneity ◽  
Familial Pancreatic Cancer ◽  
Whole Genome

Risk independence for the identification of cystic lesions in Familial Pancreatic Cancer (FPC) kindreds

Pancreatology ◽  
2017 ◽  
Vol 17 (3) ◽  
pp. S68
Author(s):  
Andrea Sheel ◽  
Sara Harrison ◽  
Ioannis Sarantitis ◽  
James Nicholson ◽  
Christopher Halloran ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cystic Lesions

scholarly journals Identification of Cystic Lesions by Secondary Screening of Familial Pancreatic Cancer (FPC) Kindreds Is Not Associated with the Stratified Risk of Cancer

2019 ◽  
Vol 114 (1) ◽  
pp. 155-164 ◽  
Author(s):  
A. R. G. Sheel ◽  
S. Harrison ◽  
I. Sarantitis ◽  
J. A. Nicholson ◽  
T. Hanna ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cystic Lesions ◽  
Secondary Screening ◽  
Risk Of Cancer

Update on familial pancreatic cancer

2001 ◽  
Vol 3 (2) ◽  
pp. 121-128 ◽  
Author(s):  
Henry T. Lynch ◽  
Randall E. Brand ◽  
Carolyn A. Deters ◽  
Ramon M. Fusaro
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer

Risk factors for the development of pancreatic cancer in familial pancreatic cancer kindreds

2003 ◽  
Vol 124 (5) ◽  
pp. 1292-1299 ◽  
Author(s):  
Stephen J Rulyak ◽  
Albert B Lowenfels ◽  
Patrick Maisonneuve ◽  
Teresa A Brentnall
Keyword(s):  
Risk Factors ◽  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer
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