Tags

Type your tag names separated by a space and hit enter

MEN1 gene analysis in sporadic adrenocortical neoplasms.
J Clin Endocrinol Metab. 1999 Jan; 84(1):216-9.JC

Abstract

Adrenocortical tumors occur as sporadic tumors, as part of the multiple endocrine neoplasia type 1 (MEN1) syndrome or as part of other hereditary disorders. We recently cloned the MEN1 gene, a tumor-suppressor gene located on chromosome 11q13. Subsequently, we showed that sequential somatic inactivation of both alleles of the MEN1 gene contributes to the development of some sporadic endocrine neoplasms (parathyroid, enteropancreatic neuroendocrine, bronchial carcinoid, and pituitary tumors). We now studied whether somatic inactivation of the MEN1 gene contributes to the pathogenesis of sporadic adrenocortical neoplasms. Seven adrenocortical carcinomas, 2 adrenocortical carcinoma cell lines, and 11 aldosterone-secreting, 8 cortisol-secreting, and 5 nonsecreting benign adrenocortical tumors were studied. Seven tumors (5 of 5 carcinomas, 2 of 21 nonsecreting benign adenomas; P < 0.001) exhibited loss of heterozygosity on 11q13. All 33 tumors and cell lines were screened for mutation throughout the MEN1 open-reading frame and adjacent splice junctions. None exhibited a mutation within the MEN1-coding region. We conclude that somatic MEN1 mutation within the MEN1-coding region does not occur commonly in sporadic adrenocortical tumors, although the majority of adrenocortical carcinomas exhibit 11q13 loss of heterozygosity.

Authors+Show Affiliations

Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892-1802, USA. christinah@bdg10.niddk.nih.govNo 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, Non-U.S. Gov't

Language

eng

PubMed ID

9920087

Citation

Heppner, C, et al. "MEN1 Gene Analysis in Sporadic Adrenocortical Neoplasms." The Journal of Clinical Endocrinology and Metabolism, vol. 84, no. 1, 1999, pp. 216-9.
Heppner C, Reincke M, Agarwal SK, et al. MEN1 gene analysis in sporadic adrenocortical neoplasms. J Clin Endocrinol Metab. 1999;84(1):216-9.
Heppner, C., Reincke, M., Agarwal, S. K., Mora, P., Allolio, B., Burns, A. L., Spiegel, A. M., & Marx, S. J. (1999). MEN1 gene analysis in sporadic adrenocortical neoplasms. The Journal of Clinical Endocrinology and Metabolism, 84(1), 216-9.
Heppner C, et al. MEN1 Gene Analysis in Sporadic Adrenocortical Neoplasms. J Clin Endocrinol Metab. 1999;84(1):216-9. PubMed PMID: 9920087.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - MEN1 gene analysis in sporadic adrenocortical neoplasms. AU - Heppner,C, AU - Reincke,M, AU - Agarwal,S K, AU - Mora,P, AU - Allolio,B, AU - Burns,A L, AU - Spiegel,A M, AU - Marx,S J, PY - 1999/1/27/pubmed PY - 1999/1/27/medline PY - 1999/1/27/entrez SP - 216 EP - 9 JF - The Journal of clinical endocrinology and metabolism JO - J Clin Endocrinol Metab VL - 84 IS - 1 N2 - Adrenocortical tumors occur as sporadic tumors, as part of the multiple endocrine neoplasia type 1 (MEN1) syndrome or as part of other hereditary disorders. We recently cloned the MEN1 gene, a tumor-suppressor gene located on chromosome 11q13. Subsequently, we showed that sequential somatic inactivation of both alleles of the MEN1 gene contributes to the development of some sporadic endocrine neoplasms (parathyroid, enteropancreatic neuroendocrine, bronchial carcinoid, and pituitary tumors). We now studied whether somatic inactivation of the MEN1 gene contributes to the pathogenesis of sporadic adrenocortical neoplasms. Seven adrenocortical carcinomas, 2 adrenocortical carcinoma cell lines, and 11 aldosterone-secreting, 8 cortisol-secreting, and 5 nonsecreting benign adrenocortical tumors were studied. Seven tumors (5 of 5 carcinomas, 2 of 21 nonsecreting benign adenomas; P < 0.001) exhibited loss of heterozygosity on 11q13. All 33 tumors and cell lines were screened for mutation throughout the MEN1 open-reading frame and adjacent splice junctions. None exhibited a mutation within the MEN1-coding region. We conclude that somatic MEN1 mutation within the MEN1-coding region does not occur commonly in sporadic adrenocortical tumors, although the majority of adrenocortical carcinomas exhibit 11q13 loss of heterozygosity. SN - 0021-972X UR - https://www.unboundmedicine.com/medline/citation/9920087/MEN1_gene_analysis_in_sporadic_adrenocortical_neoplasms_ L2 - https://academic.oup.com/jcem/article-lookup/doi/10.1210/jcem.84.1.5388 DB - PRIME DP - Unbound Medicine ER -