Pediatric adrenocortical tumors: what they can tell us on adrenal development and comparison with adult adrenal tumors

Frontiers in Endocrinology
Enzo Lalli, Bonald C Figueiredo

Abstract

Adrenocortical tumors (ACT) in children are very rare and are most frequently diagnosed in the context of the Li-Fraumeni syndrome, a multiple cancer syndrome linked to germline mutations of the tumor suppressor gene TP53 with loss of heterozygosity in the tumors. A peak of children ACT incidence is present in the states of southern Brazil, where they are linked to the high prevalence in the population of a specific TP53 mutation (R337H). Children ACT have specific features distinguishing them from adult tumors in their pathogenetic mechanisms, genomic profiles, and prognosis. Epidemiological and molecular evidence suggests that in most cases they are derived from the fetal adrenal.

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Citations

Dec 19, 2015·Molecular and Cellular Endocrinology·Kaitlin J BashamGary D Hammer
Jun 16, 2016·Molecular and Cellular Endocrinology·Alexandre NaccacheMireille Castanet
Nov 17, 2017·Endocrine-related Cancer·Enzo Lalli, Michaela Luconi
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Feb 9, 2017·Journal of Pediatric Endocrinology & Metabolism : JPEM·Andréa Farias de Melo-LeiteJorge Elias
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Dec 2, 2021·The Journal of Clinical Endocrinology and Metabolism·Michael R ClayKatja Kiseljak-Vassiliades
Jan 1, 2022·Pediatric Blood & Cancer·Luciana Chain VeronezCarlos Alberto Scrideli

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Methods Mentioned

BETA
chromosomal aberrations
exome sequencing

Software Mentioned

miRNome
IPACTR

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