Familial neuroendocrine tumors as a model of hereditary cancer

Research output: Contribution to journalReview article

4 Citations (Scopus)

Abstract

Familial neuroendocrine tumors are reviewed. The most dramatic advances have been in the application of molecular genetic techniques to define the affected genes and to develop predictive testing for patients with multiple endocrine neoplasia syndromes. Germline mutations at specific loci of the RET proto-oncogene have been demonstrated in patients with multiple endocrine neoplasia types IIA, IIB, and familial medullary thyroid carcinoma not associated with multiple endocrine neoplasia. This has led to direct DNA testing for these mutations in patients at risk for these syndromes. The approach to predictive testing, diagnosis, and early treatment of these patients is discussed as a model for the approach to hereditary cancers. Linkage testing with DNA markers is still required for patients with multiple endocrine neoplasia type I because the responsible gene has not yet been isolated. Efforts to clarify the etiologies of other familial neuroendocrine tumors not associated with multiple endocrine neoplasia continue. Familial pheochromocytoma, neuroblastoma, and carcinoid also are reviewed. The use of molecular genetic techniques as a powerful tool for the early identification and treatment of susceptible individuals is emphasized.

Original languageEnglish
Pages (from-to)45-54
Number of pages10
JournalCurrent Opinion in Oncology
Volume9
Issue number1
StatePublished - 1997

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Neuroendocrine Tumors
Multiple Endocrine Neoplasia
Genetic Techniques
Neoplasms
Molecular Biology
Multiple Endocrine Neoplasia Type 1
Proto-Oncogenes
Germ-Line Mutation
Carcinoid Tumor
Neuroblastoma
Genetic Markers
Genes
Mutation
DNA
Therapeutics

All Science Journal Classification (ASJC) codes

  • Cancer Research

Cite this

Familial neuroendocrine tumors as a model of hereditary cancer. / Anderson, Robert J.; Lynch, Henry T.

In: Current Opinion in Oncology, Vol. 9, No. 1, 1997, p. 45-54.

Research output: Contribution to journalReview article

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