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Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1.
N Engl J Med. 1989 Jul 27; 321(4):213-8.NEJM

Abstract

Familial multiple endocrine neoplasia type 1 (MEN-1) is characterized by tumors of the parathyroids, endocrine pancreas, and anterior pituitary. Since the gene associated with MEN-1, located on chromosome 11 (11q13), may normally inhibit tumor proliferation, tumors could arise from inactivation of one or both of the alleles. However, parathyroid tumors in patients with MEN-1 have been considered to result from polyclonal hyperplasia. Using genetic probes, we tested parathyroid tumors for a monoclonal component, represented by a loss of alleles at any of eight loci along chromosome 11. Ten of 16 tumors from 14 patients with familial MEN-1 had losses of alleles from chromosome 11. Tumors with losses were larger than those without (1.6 vs. 0.2 g; P less than 0.002), suggesting that a monoclonal adenoma may develop after a phase of polyclonal hyperplasia. In 7 of 10 tumors, the subregion of loss was less than the full length of chromosome 11 but always included one copy of the MEN-1 locus. Of 34 sporadic adenomas from patients without MEN-1, 9 showed similar allelic losses in chromosome 11; in 7 the losses included the apparent MEN-1 locus. We conclude that many "hyperplastic" parathyroid tumors in familial MEN-1 are in fact monoclonal and may progress or even begin to develop by inactivation of the MEN-1 gene (at 11q13) in a precursor cell. Some sporadic adenomas have allelic losses on chromosome 11, which may also involve the MEN-1 gene.

Authors+Show Affiliations

Molecular Pathophysiology Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Md.No affiliation info availableNo affiliation info availableNo 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

Language

eng

PubMed ID

2568586

Citation

Friedman, E, et al. "Clonality of Parathyroid Tumors in Familial Multiple Endocrine Neoplasia Type 1." The New England Journal of Medicine, vol. 321, no. 4, 1989, pp. 213-8.
Friedman E, Sakaguchi K, Bale AE, et al. Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1. N Engl J Med. 1989;321(4):213-8.
Friedman, E., Sakaguchi, K., Bale, A. E., Falchetti, A., Streeten, E., Zimering, M. B., Weinstein, L. S., McBride, W. O., Nakamura, Y., & Brandi, M. L. (1989). Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1. The New England Journal of Medicine, 321(4), 213-8.
Friedman E, et al. Clonality of Parathyroid Tumors in Familial Multiple Endocrine Neoplasia Type 1. N Engl J Med. 1989 Jul 27;321(4):213-8. PubMed PMID: 2568586.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1. A1 - Friedman,E, AU - Sakaguchi,K, AU - Bale,A E, AU - Falchetti,A, AU - Streeten,E, AU - Zimering,M B, AU - Weinstein,L S, AU - McBride,W O, AU - Nakamura,Y, AU - Brandi,M L, PY - 1989/7/27/pubmed PY - 1989/7/27/medline PY - 1989/7/27/entrez SP - 213 EP - 8 JF - The New England journal of medicine JO - N Engl J Med VL - 321 IS - 4 N2 - Familial multiple endocrine neoplasia type 1 (MEN-1) is characterized by tumors of the parathyroids, endocrine pancreas, and anterior pituitary. Since the gene associated with MEN-1, located on chromosome 11 (11q13), may normally inhibit tumor proliferation, tumors could arise from inactivation of one or both of the alleles. However, parathyroid tumors in patients with MEN-1 have been considered to result from polyclonal hyperplasia. Using genetic probes, we tested parathyroid tumors for a monoclonal component, represented by a loss of alleles at any of eight loci along chromosome 11. Ten of 16 tumors from 14 patients with familial MEN-1 had losses of alleles from chromosome 11. Tumors with losses were larger than those without (1.6 vs. 0.2 g; P less than 0.002), suggesting that a monoclonal adenoma may develop after a phase of polyclonal hyperplasia. In 7 of 10 tumors, the subregion of loss was less than the full length of chromosome 11 but always included one copy of the MEN-1 locus. Of 34 sporadic adenomas from patients without MEN-1, 9 showed similar allelic losses in chromosome 11; in 7 the losses included the apparent MEN-1 locus. We conclude that many "hyperplastic" parathyroid tumors in familial MEN-1 are in fact monoclonal and may progress or even begin to develop by inactivation of the MEN-1 gene (at 11q13) in a precursor cell. Some sporadic adenomas have allelic losses on chromosome 11, which may also involve the MEN-1 gene. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/2568586/Clonality_of_parathyroid_tumors_in_familial_multiple_endocrine_neoplasia_type_1_ L2 - https://www.nejm.org/doi/10.1056/NEJM198907273210402?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -