Cited by 115Adrenal and Paraganglionic TumorsChromatin Remodeling and CancerAdrenal Hormones and Disorders

Somatic TP53 Mutations Are Relatively Rare among Adrenocortical Cancers with the Frequent 17p13 Loss of Heterozygosity

Libè, Rossella, Groussin, Lionel, Tissier, Frédérique, Elie, Caroline, René-Corail, Fernande, Fratticci, Amato, Jullian, Eric, Beck-Peccoz, Paolo +3 more·2007·Clinical Cancer Research

Abstract

PURPOSE: Allelic losses [loss of heterozygosity (LOH)] at the 17p13 locus are frequent (85%) in adrenocortical cancers. The tumor suppressor gene TP53 is located at 17p13. The aim of the study was to determine the frequency of TP53 somatic inactivating mutations in adrenocortical tumors with 17p13 LOH and their clinico-biological correlations. EXPERIMENTAL DESIGN: TP53 somatic mutations, intragenic LOH (VNTR1 marker), and p53 overexpression were studied in 36 adrenocortical tumors with 17p13 LOH determined by Southern blot. RESULTS: TP53 mutations were detected in 33% of the tumors, and VNTR1 LOH was present in 44% of the cases and did not always correlate with the presence of a TP53 mutation. Only the TP53-mutant tumors exhibit a strong nuclear immunoreactivity. TP53-mutant tumors were significantly larger than wild-type TP53 tumors (median tumor weight: 640 versus 185 g; P=0.02), were associated with a more advanced stage of tumor progression (MacFarlane stage IV; P=0.01), and had a shorter disease-free survival (P=0.03). CONCLUSIONS: The finding that only a minority of adrenocortical tumors with 17p13 LOH had either a VNTR1 LOH or a TP53 mutation indicates that TP53 might not be the only or major tumor suppressor gene at 17p13 involved in adrenocortical cancer progression. We suggest that a genetic instability of the 17p13 region, occurring early in adrenocortical cancer development, involves various genes located in this region. TP53 might be only one of them, and its alteration by the occurrence of inactivating mutation is associated with the development of more aggressive tumors.

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Crossref · 10.1158/1078-0432.ccr-06-2085

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  • Only one-third of adrenocortical carcinomas with loss of heterozygosity of chromosome 17p13 have a mutation of TP53.

    “However, only one-third of these ACCs have a mutation of TP53 (163).”

    From Adrenal incidentaloma (2020) · cites this paper as [163]

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Authors
Libè, R., Groussin, L., Tissier, F., Elie, C., René-Corail, F., Fratticci, A., Jullian, E., Beck-Peccoz, P., Bertagna, X., Gicquel, C., & Bertherat, J.
Affiliations
  • 1Institut National de la Sante et de la Recherche Medicale U567;
  • Centre National de la Recherche Scientifique (FR)
  • Hôpital Cochin (FR)
  • 10Institute of Endocrine Sciences, Ospedale Maggiore of Milan, Instituto di Ricovero e Cura a Carattere Scientifico, University of Milan, Milan, Italy
  • Hôpital Armand-Trousseau (FR)
Venue
Clinical Cancer Research
Published
2007-02-01
Volume
13
Issue
3
Pages
844-850
ISSN
  • 1078-0432 (ISSN-L)
  • 1557-3265
OpenAlex
W2103134006
Cited by
115
References
50
Citations by year
2026: 1 · 2025: 1 · 2024: 3 · 2023: 3 · 2022: 6 · 2021: 4 · 2020: 7 · 2019: 6 · 2018: 9 · 2017: 5 · 2016: 4 · 2015: 9 · 2014: 7 · 2013: 8 · 2012: 8
FWCI
7.31
Type
journal article
Language
en
Open access
closed
Locations
  • Clinical Cancer Research (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Chromatin Remodeling and Cancer
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Keywords
TP53 mutation, adrenocortical carcinoma, adrenocortical tumors, p53 overexpression, tumor suppressor, genomic instability, tumor progression, disease-free survival, tumor size
MeSH terms
Adrenal Cortex Neoplasms, Adult, Alleles, Chromosomes, Human, Pair 17, Female, Humans, Immunohistochemistry, Male, Middle Aged, Mutation, RNA, Neoplasm, Genes, p53, Disease Progression, Minisatellite Repeats, Loss of Heterozygosity

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