Highly penetrant hereditary cancer syndromes.

@article{citeulike:2789804, abstract = {The past two decades have brought many important advances in our understanding of the hereditary susceptibility to cancer. Approximately 5-10\% of all cancers are inherited, the majority in an autosomal dominant manner with incomplete penetrance. While this is a small fraction of the overall cancer burden worldwide, the molecular genetic discoveries that have resulted from the study of families with heritable cancer have not only changed the way these families are counselled and managed, but have shed light on molecular regulatory pathways important in sporadic tumour development as well. In this review, we consider 10 of the more highly penetrant cancer syndromes, with emphasis on those predisposing to breast, colon, and/or endocrine neoplasia. We discuss the prevalence, penetrance, and tumour spectrum associated with these syndromes, as well as their underlying genetic defects.}, address = {Clinical Cancer Genetics Program, Comprehensive Cancer Center, Division of Human Genetics, Department of Internal Medicine, The Ohio State University, Columbus 43221, USA. nagy-1@medctr.osu.edu}, author = {Nagy, R. and Sweet, K. and Eng, C. }, citeulike-article-id = {2789804}, doi = {10.1038/sj.onc.1207714}, issn = {0950-9232}, journal = {Oncogene}, keywords = {cancer}, month = {August}, number = {38}, pages = {6445--6470}, posted-at = {2008-05-12 16:40:47}, priority = {0}, title = {Highly penetrant hereditary cancer syndromes.}, url = {http://dx.doi.org/10.1038/sj.onc.1207714}, volume = {23}, year = {2004} }

TY - JOUR ID - citeulike:2789804 L3 - citeulike-article-id:2789804 TI - Highly penetrant hereditary cancer syndromes. JF - Oncogene VL - 23 IS - 38 SP - 6445 EP - 6470 AD - Clinical Cancer Genetics Program, Comprehensive Cancer Center, Division of Human Genetics, Department of Internal Medicine, The Ohio State University, Columbus 43221, USA. nagy-1@medctr.osu.edu SN - 0950-9232 N2 - The past two decades have brought many important advances in our understanding of the hereditary susceptibility to cancer. Approximately 5-10% of all cancers are inherited, the majority in an autosomal dominant manner with incomplete penetrance. While this is a small fraction of the overall cancer burden worldwide, the molecular genetic discoveries that have resulted from the study of families with heritable cancer have not only changed the way these families are counselled and managed, but have shed light on molecular regulatory pathways important in sporadic tumour development as well. In this review, we consider 10 of the more highly penetrant cancer syndromes, with emphasis on those predisposing to breast, colon, and/or endocrine neoplasia. We discuss the prevalence, penetrance, and tumour spectrum associated with these syndromes, as well as their underlying genetic defects. KW - cancer AU - Nagy, R AU - Sweet, K AU - Eng, C PY - 2004/08/23/ UR - http://dx.doi.org/10.1038/sj.onc.1207714 ER -