Format

Send to

Choose Destination
Eur J Cancer. 2015 Sep;51(14):1911-7. doi: 10.1016/j.ejca.2015.07.004. Epub 2015 Jul 23.

PanGen-Fam: Spanish registry of hereditary pancreatic cancer.

Author information

1
Medical Oncology Department, Ramon y Cajal University Hospital, IRYCIS, Madrid, Spain.
2
Genetic and Molecular Epidemiology Group, Spanish National Cancer Research Centre (CNIO), Madrid, Spain.
3
Molecular Oncogenetics Unit, Institute of Medical and Molecular Genetics, La Paz Hospital, Madrid, Spain.
4
Digestive Department, Ramon y Cajal University Hospital, IRYCIS, Madrid, Spain.
5
Pathology Department, Ramon y Cajal University Hospital, IRYCIS, Madrid, Spain.
6
Radiology Department, Ramon y Cajal University Hospital, IRYCIS, Madrid, Spain.
7
Surgery Department, Ramon y Cajal University Hospital, IRYCIS, Madrid, Spain.
8
Epithelial Carcinogenesis Group, Spanish National Cancer Research Centre (CNIO), Madrid, Spain; Department de Ciències Experimentals i de la Salut, Universitat Pompeu Fabra, Barcelona, Spain.

Abstract

PURPOSE:

To describe the organisation of the registry and the preliminary results in terms of characteristics of high-risk pancreatic ductal adenocarcinoma (PDAC) families recruited to date and findings of the screening programme. To compare early onset sporadic cases (⩽50 years), sporadic cases (>50 years) and cases with family history of cancer, for PDAC possible risk factors.

METHODS/PATIENTS:

Families with hereditary cancer syndromes predisposing to PDAC were recruited from two main sources: Spanish hospitals participating in PanGenEU, a pan-European multicentre case-control study, and their genetic counseling unit. Individuals at high-risk of PDAC were enrolled into a screening programme, consisting of Endoscopic ultrasound, computerised tomography, magnetic resonance imaging. Genetic testing of candidate genes was offered according to each patient's risk.

RESULTS:

Among 577 consecutive PDAC cases, recruited via PanGenEU, 36 (6%) had ⩾2 first-degree relative with PDAC: Familial pancreatic cancer (FPC). So far PanGen-Fam has recruited 42 high-risk PDAC families; 25 (60%) had FPC. Five index cases with cancer were positive for BRCA2 and one for BRCA1 germline mutations. In the second year of prospective PDAC screening, one neuroendocrine tumour and a high-grade dysplasia lesion suspicious of carcinoma were diagnosed among 41 high-risk individuals. Furthermore EUS detected chronic-pancreatitis-like parenchymal changes in 15 patients.

CONCLUDING STATEMENT:

The identification and recruitment of PDAC high-risk families into the PanGen-Fam registry provides an opportunity to detect early onset cancer and precursor pancreatic cancer lesions at a potentially curative stage and to increase the knowledge of the natural history of the disease.

KEYWORDS:

Early onset PDAC; Familial pancreatic cancer; High-risk individuals; Pancreatic ductal adenocarcinoma; Precursor lesions; Screening; Tumour hereditary syndromes

PMID:
26212471
DOI:
10.1016/j.ejca.2015.07.004
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center