Genetic predisposition to childhood cancer: A manuscript that describes the main tumor predispositions in childhood

· TGI - Cancer, TGI - Gestation

[Article in French]

Arch Pediatr. 2012 Aug;19(8):863-75. Epub 2012 Jul 15.

Authors: Abadie CGauthier-Villars MSirvent NCoupier I.


Unité d’oncogénétique, CRLC Val-d’Aurelle, 34298 Montpellier cedex 05, France; Service de génétique médicale, CHU de Rennes, 35033 Rennes cedex 9, France.


Tumor predisposition in children is rare, accounting for approximately 10% of all cancers in childhood.

Tumor predisposition involves very rare tumors such as

Pleuropulmonary blastoma,

Adrenocortical carcinoma,


Rhabdoid tumors,

Optic pathway glioma,





Neuroblastoma, and


The identification of these predispositions is important for improved management for both the child and relatives.

Prenatal and preimplantation genetic diagnosis are options that could be considered for young parents in a perspective of future pregnancies.

This manuscript describes the main tumor predispositions in childhood. From each histological subtype, the different diagnosis directions are discussed in view of these main tumor predispositions.

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