Hereditary breast cancer and family cancer syndromes

Henry T. Lynch, Jane Lynch, Theresa Conway, Patrice Watson, Jean Feunteum, Gilbert Lenoir, Steven Narod, Robert Joseph Fitzgibbons

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

Hereditary breast cancer (HBC) shows extant clinical and genetic heterogeneity. Clinically one finds the onset of breast cancer at an early age, an excess of bilaterality, and patterns of multiple primary cancer such as combinations of breast and ovarian carcinoma in the hereditary breast-ovarian cancer (HBOC) syndrome. In addition to HBOC, one sees a variety of putative breast cancer-prone genotypes inclusive of hereditary site-specific breast cancer, and the Li-Frameni (SBLA) syndrome that is characterized by cancers involving all three germinal layers including sarcomas, brain tumors, leukemia, lymphoma, and adrenal cortical carcinoma in addition to often markedly early-onset breast cancer. Breast cancer is also associated with autosomal dominantly inherited Cowden's disease and autosomal recessively inherited ataxia-telangiectasia. Examples of pedigrees depicting clincal examples of these several HBC syndromes are presented in order to describe HBC's heterogeneity. The recent identification of the BRCA1 gene in early-onset hereditary sitespecific breast cancer and the HBOC syndrome has led to new challenges for the genetic counselor. We review genetic counseling, which embraces surveillance and management recommendations that are responsive to the natural history of HBC and address the concept for future development of centers of expertise for HBC in the interest of improving cancer control.

Original languageEnglish
Pages (from-to)21-31
Number of pages11
JournalWorld Journal of Surgery
Volume18
Issue number1
DOIs
StatePublished - Jan 1994

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Breast Neoplasms
Neoplasms
Hereditary Breast and Ovarian Cancer Syndrome
Hereditary Neoplastic Syndromes
Multiple Hamartoma Syndrome
BRCA1 Gene
Adrenocortical Carcinoma
Ataxia Telangiectasia
Genetic Heterogeneity
Genetic Counseling
Pedigree
Natural History
Brain Neoplasms
Sarcoma
Ovarian Neoplasms
Lymphoma
Leukemia
Genotype

All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

Hereditary breast cancer and family cancer syndromes. / Lynch, Henry T.; Lynch, Jane; Conway, Theresa; Watson, Patrice; Feunteum, Jean; Lenoir, Gilbert; Narod, Steven; Fitzgibbons, Robert Joseph.

In: World Journal of Surgery, Vol. 18, No. 1, 01.1994, p. 21-31.

Research output: Contribution to journalArticle

Lynch, HT, Lynch, J, Conway, T, Watson, P, Feunteum, J, Lenoir, G, Narod, S & Fitzgibbons, RJ 1994, 'Hereditary breast cancer and family cancer syndromes', World Journal of Surgery, vol. 18, no. 1, pp. 21-31. https://doi.org/10.1007/BF00348188
Lynch HT, Lynch J, Conway T, Watson P, Feunteum J, Lenoir G et al. Hereditary breast cancer and family cancer syndromes. World Journal of Surgery. 1994 Jan;18(1):21-31. https://doi.org/10.1007/BF00348188
Lynch, Henry T. ; Lynch, Jane ; Conway, Theresa ; Watson, Patrice ; Feunteum, Jean ; Lenoir, Gilbert ; Narod, Steven ; Fitzgibbons, Robert Joseph. / Hereditary breast cancer and family cancer syndromes. In: World Journal of Surgery. 1994 ; Vol. 18, No. 1. pp. 21-31.
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