Published in Hered Cancer Clin Pract on June 28, 2017
FOLFIRINOX versus gemcitabine for metastatic pancreatic cancer. N Engl J Med (2011) 24.32
Increased survival in pancreatic cancer with nab-paclitaxel plus gemcitabine. N Engl J Med (2013) 16.24
Genetic heterogeneity and penetrance analysis of the BRCA1 and BRCA2 genes in breast cancer families. The Breast Cancer Linkage Consortium. Am J Hum Genet (1998) 15.09
PD-1 Blockade in Tumors with Mismatch-Repair Deficiency. N Engl J Med (2015) 13.08
DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science (2011) 10.26
Exomic sequencing identifies PALB2 as a pancreatic cancer susceptibility gene. Science (2009) 7.39
Ataxia-telangiectasia: from a rare disorder to a paradigm for cell signalling and cancer. Nat Rev Mol Cell Biol (2008) 6.62
von Hippel-Lindau disease. Lancet (2003) 6.20
Breast-cancer risk in families with mutations in PALB2. N Engl J Med (2014) 4.97
Beyond Li Fraumeni Syndrome: clinical characteristics of families with p53 germline mutations. J Clin Oncol (2009) 4.84
Cancer Statistics, 2017. CA Cancer J Clin (2017) 4.77
Olaparib maintenance therapy in patients with platinum-sensitive relapsed serous ovarian cancer: a preplanned retrospective analysis of outcomes by BRCA status in a randomised phase 2 trial. Lancet Oncol (2014) 4.65
Genetic aspects of adenomatosis of endocrine glands. Am J Med (1954) 4.60
Ashkenazi Jewish population frequencies for common mutations in BRCA1 and BRCA2. Nat Genet (1996) 4.36
Prediction of germline mutations and cancer risk in the Lynch syndrome. JAMA (2006) 4.34
Hereditary pancreatitis and the risk of pancreatic cancer. International Hereditary Pancreatitis Study Group. J Natl Cancer Inst (1997) 4.23
Increased risk of pancreatic cancer in melanoma-prone kindreds with p16INK4 mutations. N Engl J Med (1995) 4.10
Whole genomes redefine the mutational landscape of pancreatic cancer. Nature (2015) 4.01
Review of the Lynch syndrome: history, molecular genetics, screening, differential diagnosis, and medicolegal ramifications. Clin Genet (2009) 3.99
Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res (2006) 3.81
Cancer risk estimates for BRCA1 mutation carriers identified in a risk evaluation program. J Natl Cancer Inst (2002) 3.65
The NF1 tumor suppressor critically regulates TSC2 and mTOR. Proc Natl Acad Sci U S A (2005) 3.49
Prospective risk of pancreatic cancer in familial pancreatic cancer kindreds. Cancer Res (2004) 3.31
ATM mutations in patients with hereditary pancreatic cancer. Cancer Discov (2011) 3.27
Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol (2008) 2.97
Clinical and genetic characteristics of hereditary pancreatitis in Europe. Clin Gastroenterol Hepatol (2004) 2.82
Geographical variation in the penetrance of CDKN2A mutations for melanoma. J Natl Cancer Inst (2002) 2.70
Identification of the multiple endocrine neoplasia type 1 (MEN1) gene. The European Consortium on MEN1. Hum Mol Genet (1997) 2.45
Familial adenomatous polyposis (FAP): frequency, penetrance, and mutation rate. Hum Mutat (1994) 2.44
High-risk melanoma susceptibility genes and pancreatic cancer, neural system tumors, and uveal melanoma across GenoMEL. Cancer Res (2006) 2.40
The natural history of hereditary pancreatitis: a national series. Gut (2008) 2.33
Risk of pancreatic cancer in families with Lynch syndrome. JAMA (2009) 2.32
Tobacco and the risk of pancreatic cancer: a review and meta-analysis. Langenbecks Arch Surg (2008) 2.29
Diabetes, antidiabetic medications, and pancreatic cancer risk: an analysis from the International Pancreatic Cancer Case-Control Consortium. Ann Oncol (2014) 2.24
Familial adenomatous polyposis. Orphanet J Rare Dis (2009) 2.21
Pancreatic involvement in von Hippel-Lindau disease. The Groupe Francophone d'Etude de la Maladie de von Hippel-Lindau. Gastroenterology (2000) 2.16
Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer (2008) 2.11
The prevalence of BRCA2 mutations in familial pancreatic cancer. Cancer Epidemiol Biomarkers Prev (2007) 2.03
Differences in survival by histologic type of pancreatic cancer. Cancer Epidemiol Biomarkers Prev (2005) 2.03
PALB2 mutations in European familial pancreatic cancer families. Clin Genet (2010) 1.99
BRCA1 up-regulation is associated with repair-mediated resistance to cis-diamminedichloroplatinum(II). Cancer Res (1998) 1.97
Risk of developing pancreatic cancer in families with familial atypical multiple mole melanoma associated with a specific 19 deletion of p16 (p16-Leiden). Int J Cancer (2000) 1.90
Use of the national institutes of health criteria for diagnosis of neurofibromatosis 1 in children. Pediatrics (2000) 1.79
Prevalence of germline mutations in cancer predisposition genes in patients with pancreatic cancer. Gastroenterology (2014) 1.78
The role of PARP in DNA repair and its therapeutic exploitation. Br J Cancer (2011) 1.71
Microadenomatosis of the endocrine pancreas in patients with and without the multiple endocrine neoplasia type 1 syndrome. Am J Surg Pathol (2006) 1.71
Germline BRCA Mutations in a Large Clinic-Based Cohort of Patients With Pancreatic Adenocarcinoma. J Clin Oncol (2015) 1.60
Molecular pathology of ataxia telangiectasia. J Clin Pathol (2005) 1.59
Peutz-Jeghers syndrome: diagnostic and therapeutic approach. World J Gastroenterol (2009) 1.57
TP53 germline mutation testing in 180 families suspected of Li-Fraumeni syndrome: mutation detection rate and relative frequency of cancers in different familial phenotypes. J Med Genet (2010) 1.54
Von Recklinghausen's neurofibromatosis: neurofibromatosis type 1. Lancet (2003) 1.51
Li-Fraumeni syndrome: cancer risk assessment and clinical management. Nat Rev Clin Oncol (2014) 1.42
Familial and second primary pancreatic cancers: a nationwide epidemiologic study from Sweden. Int J Cancer (2003) 1.39
Exonic STK11 deletions are not a rare cause of Peutz-Jeghers syndrome. J Med Genet (2006) 1.39
Family history is a significant risk factor for pancreatic cancer: results from a systematic review and meta-analysis. Fam Cancer (2008) 1.36
Evidence for a major gene influencing risk of pancreatic cancer. Genet Epidemiol (2002) 1.36
The incidence of pancreatic cancer in BRCA1 and BRCA2 mutation carriers. Br J Cancer (2012) 1.34
Epidemiology data on 108 MEN 1 patients from the GTE with isolated nonfunctioning tumors of the pancreas. Ann Surg (2006) 1.34
Familial pancreatic cancer. Ann Oncol (1999) 1.33
Increased risk of cancer other than melanoma in CDKN2A founder mutation (p16-Leiden)-positive melanoma families. Clin Cancer Res (2008) 1.32
Cancer risk in heterozygotes for ataxia-telangiectasia. Int J Cancer (2001) 1.32
Loss of BRCA1 function increases the antitumor activity of cisplatin against human breast cancer xenografts in vivo. Cancer Biol Ther (2009) 1.32
Alcohol consumption and pancreatic cancer: a pooled analysis in the International Pancreatic Cancer Case-Control Consortium (PanC4). Ann Oncol (2011) 1.29
Risk factors associated with earlier age of onset in familial pancreatic carcinoma. Cancer (2004) 1.20
Neuroendocrine tumor disease: an evolving landscape. Endocr Relat Cancer (2012) 1.17
Pancreatitis-diabetes-pancreatic cancer: summary of an NIDDK-NCI workshop. Pancreas (2013) 1.15
Epidemiology of and risk factors for pancreatic cancer. Surg Oncol Clin N Am (1998) 1.14
Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics (2005) 1.09
Cancer risk associated with STK11/LKB1 germline mutations in Peutz-Jeghers syndrome patients: results of an Italian multicenter study. Dig Liver Dis (2013) 1.06
Predictive biomarkers for checkpoint inhibitor-based immunotherapy. Lancet Oncol (2016) 1.05
Hyperparathyroidism presenting as the first lesion in multiple endocrine neoplasia type 1. Am J Med (1987) 1.03
Medullary carcinoma of the pancreas in a man with hereditary nonpolyposis colorectal cancer due to a mutation of the MSH2 mismatch repair gene. Hum Pathol (2006) 1.02
Are neuroendocrine tumours a feature of tuberous sclerosis? A systematic review. Endocr Relat Cancer (2008) 0.99
Genetics and Genetic Testing in Pancreatic Cancer. Gastroenterology (2015) 0.98
BRCA1, BRCA2, PALB2, and CDKN2A mutations in familial pancreatic cancer: a PACGENE study. Genet Med (2014) 0.97
Pancreatic cancer risk in Peutz-Jeghers syndrome patients: a large cohort study and implications for surveillance. J Med Genet (2013) 0.96
Whole exome sequencing identifies multiple, complex etiologies in an idiopathic hereditary pancreatitis kindred. JOP (2012) 0.96
Absence of germline BRCA1 mutations in familial pancreatic cancer patients. Cancer Biol Ther (2009) 0.95
Hereditary melanoma: Update on syndromes and management: Emerging melanoma cancer complexes and genetic counseling. J Am Acad Dermatol (2016) 0.94
Hamartomatous polyposis syndromes: a review. Orphanet J Rare Dis (2014) 0.90
Whole Genome Sequencing Defines the Genetic Heterogeneity of Familial Pancreatic Cancer. Cancer Discov (2015) 0.90
Prevalence of Pathogenic Mutations in Cancer Predisposition Genes among Pancreatic Cancer Patients. Cancer Epidemiol Biomarkers Prev (2015) 0.89
Genome-wide sequencing to identify the cause of hereditary cancer syndromes: with examples from familial pancreatic cancer. Cancer Lett (2012) 0.85
Well-differentiated pancreatic neuroendocrine carcinoma in tuberous sclerosis--case report and review of the literature. Am J Surg Pathol (2012) 0.84
[Hereditary multiple endocrine neoplasia. New genetic data and clinical applications in type 1 multiple endocrine neoplasia]. Presse Med (1995) 0.84
Familial pancreatic cancer: Concept, management and issues. World J Gastroenterol (2017) 0.82
Cationic trypsinogen mutations and pancreatitis. Gastroenterol Clin North Am (2004) 0.81
Adenocarcinoma of pancreas and duodenum associated with cutaneous neurofibromatosis. Am J Med (1987) 0.81
Risk factors for pancreatic neuroendocrine tumors: a clinic-based case-control study. Pancreas (2014) 0.80
The impact of cystic fibrosis and PSTI/SPINK1 gene mutations on susceptibility to chronic pancreatitis. Clin Lab Med (2005) 0.79
European cancer mortality predictions for the year 2017, with focus on lung cancer. Ann Oncol (2017) 0.79
Prevention and screening in BRCA mutation carriers and other breast/ovarian hereditary cancer syndromes: ESMO Clinical Practice Guidelines for cancer prevention and screening. Ann Oncol (2016) 0.78
Hereditary melanoma: Update on syndromes and management: Genetics of familial atypical multiple mole melanoma syndrome. J Am Acad Dermatol (2016) 0.78
[Duodenal somatostatinoma. Anatomic/clinical study of 12 operated cases]. Ann Chir (2001) 0.78
Familial Pancreatic Adenocarcinoma. Hematol Oncol Clin North Am (2015) 0.77
Do genetic variants in the SPINK1 gene affect the level of serum PSTI? J Gastroenterol (2012) 0.77
Li-Fraumeni syndrome: a paradigm for the understanding of hereditary cancer predisposition. Br J Haematol (2016) 0.76
GEP- NETS UPDATE: Genetics of neuroendocrine tumors. Eur J Endocrinol (2016) 0.76
Role of Genomic Instability in Immunotherapy with Checkpoint Inhibitors. Anticancer Res (2016) 0.76