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Familial pancreatic cancer.
Ann Oncol 1999; 10 Suppl 4:69-73AO

Abstract

BACKGROUND

For many years anecdotal case reports have suggested that pancreatic cancer aggregates in some families.

METHODS

Two recent advances have established that this is in fact the case. First, large registries, such as the National Familial Pancreas Tumor Registry (NFPTR) at Johns Hopkins, have identified a number of families in which multiple family members have been diagnosed with pancreatic cancer. As a result, the patterns of inheritance of pancreatic cancer can now be studied on a scale not possible before. Second, advances in molecular genetic techniques make it possible to test members of these families for germline mutations in known candidate cancer causing genes. As a result, some of the genetic alterations responsible for the familial aggregation of pancreatic cancer have been identified in some families.

RESULTS

The NFPTR has enrolled 362 families in which at least one family member has been diagnosed with pancreatic cancer. These include 151 families in which at least two first-degree relatives have been diagnosed with pancreatic cancer. Analysis of these families has revealed that even second-degree relatives of patients from these families are at increased risk of developing pancreatic cancer. In addition, a number of kindreds which exhibit aggregation of cancer have been tested for germline mutations in known cancer causing genes. Germline mutations in BRCA2 have been shown to predispose to both breast and pancreatic cancer, germline mutations in p16 to melanoma and pancreatic cancer (the FAMMM syndrome), and genetic mutations in STK11/LKB1 to pancreatic cancer in patients with the Peutz-Jeghers Syndrome (PJS).

CONCLUSIONS

Pancreatic cancer aggregates in some families, and relatives of patients with pancreatic cancer have an increased risk of developing pancreatic cancer themselves. The genetic basis for the familial aggregation of pancreatic cancer has been shown to be germline mutations in known cancer causing genes in some of these families.

Authors+Show Affiliations

Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.
Review

Language

eng

PubMed ID

10436789

Citation

Hruban, R H., et al. "Familial Pancreatic Cancer." Annals of Oncology : Official Journal of the European Society for Medical Oncology, vol. 10 Suppl 4, 1999, pp. 69-73.
Hruban RH, Petersen GM, Goggins M, et al. Familial pancreatic cancer. Ann Oncol. 1999;10 Suppl 4:69-73.
Hruban, R. H., Petersen, G. M., Goggins, M., Tersmette, A. C., Offerhaus, G. J., Falatko, F., ... Kern, S. E. (1999). Familial pancreatic cancer. Annals of Oncology : Official Journal of the European Society for Medical Oncology, 10 Suppl 4, pp. 69-73.
Hruban RH, et al. Familial Pancreatic Cancer. Ann Oncol. 1999;10 Suppl 4:69-73. PubMed PMID: 10436789.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Familial pancreatic cancer. AU - Hruban,R H, AU - Petersen,G M, AU - Goggins,M, AU - Tersmette,A C, AU - Offerhaus,G J, AU - Falatko,F, AU - Yeo,C J, AU - Kern,S E, PY - 1999/8/7/pubmed PY - 1999/8/7/medline PY - 1999/8/7/entrez SP - 69 EP - 73 JF - Annals of oncology : official journal of the European Society for Medical Oncology JO - Ann. Oncol. VL - 10 Suppl 4 N2 - BACKGROUND: For many years anecdotal case reports have suggested that pancreatic cancer aggregates in some families. METHODS: Two recent advances have established that this is in fact the case. First, large registries, such as the National Familial Pancreas Tumor Registry (NFPTR) at Johns Hopkins, have identified a number of families in which multiple family members have been diagnosed with pancreatic cancer. As a result, the patterns of inheritance of pancreatic cancer can now be studied on a scale not possible before. Second, advances in molecular genetic techniques make it possible to test members of these families for germline mutations in known candidate cancer causing genes. As a result, some of the genetic alterations responsible for the familial aggregation of pancreatic cancer have been identified in some families. RESULTS: The NFPTR has enrolled 362 families in which at least one family member has been diagnosed with pancreatic cancer. These include 151 families in which at least two first-degree relatives have been diagnosed with pancreatic cancer. Analysis of these families has revealed that even second-degree relatives of patients from these families are at increased risk of developing pancreatic cancer. In addition, a number of kindreds which exhibit aggregation of cancer have been tested for germline mutations in known cancer causing genes. Germline mutations in BRCA2 have been shown to predispose to both breast and pancreatic cancer, germline mutations in p16 to melanoma and pancreatic cancer (the FAMMM syndrome), and genetic mutations in STK11/LKB1 to pancreatic cancer in patients with the Peutz-Jeghers Syndrome (PJS). CONCLUSIONS: Pancreatic cancer aggregates in some families, and relatives of patients with pancreatic cancer have an increased risk of developing pancreatic cancer themselves. The genetic basis for the familial aggregation of pancreatic cancer has been shown to be germline mutations in known cancer causing genes in some of these families. SN - 0923-7534 UR - https://www.unboundmedicine.com/medline/citation/10436789/Familial_pancreatic_cancer_ L2 - http://www.diseaseinfosearch.org/result/5551 DB - PRIME DP - Unbound Medicine ER -