Language

English

Publication Date

1-17-2025

Journal

Clinical Cancer Research

DOI

10.1158/1078-0432.CCR-24-1947

PMID

39540884

PMCID

PMC11747828

PubMedCentral® Posted Date

7-17-2025

PubMedCentral® Full Text Version

Author MSS

Abstract

Phosphate and tensin homolog hamartoma tumor syndrome, DICER1-related tumor predisposition, and tuberous sclerosis complex are rare conditions, which each increases risk for distinct spectra of benign and malignant neoplasms throughout childhood and adulthood. Surveillance considerations for each of these conditions focus on patient and family education, early detection, and multidisciplinary care. In this article, we present updated surveillance recommendations and considerations for children and adolescents with phosphate and tensin homolog hamartoma tumor syndrome, DICER1-related tumor predisposition, and tuberous sclerosis complex and provide suggestions for further research in each of these conditions.

Keywords

Humans, Ribonuclease III, Tuberous Sclerosis, Hamartoma Syndrome, Multiple, DEAD-box RNA Helicases, Child, Genetic Predisposition to Disease, PTEN Phosphohydrolase, Adolescent, Mutation, PTEN; PTEN hamartoma tumor syndrome (PHTS); DICER1; DICER1 tumor predisposition; tuberous sclerosis complex ((TSC), TSC1, TSC2); cancer surveillance; pediatric cancer predisposition; cancer risk

Published Open-Access

yes

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