Abadie C, Gauthier-Villars M, Sirvent N, Coupier I
Unité d'oncogénétique, CRLC Val-d'Aurelle, 34298 Montpellier cedex 05, France.
Arch Pediatr. 2012 Aug;19(8):863-75. doi: 10.1016/j.arcped.2012.05.017. Epub 2012 Jul 15.
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, hepatoblastoma, rhabdoid tumors, optic pathway glioma, as well as rare tumors such as retinoblastoma, medulloblastoma, nephroblastoma, or more frequent tumors such as sarcomas, neuroblastoma, and leukemias. 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.
儿童肿瘤易感性较为罕见，约占儿童所有癌症的10%。肿瘤易感性涉及非常罕见的肿瘤，如胸膜肺母细胞瘤、肾上腺皮质癌、肝母细胞瘤、横纹肌样瘤、视路胶质瘤，以及罕见肿瘤，如视网膜母细胞瘤、髓母细胞瘤、肾母细胞瘤，或更常见的肿瘤，如肉瘤、神经母细胞瘤和白血病。识别这些易感性对于改善患儿及其亲属的管理非常重要。从未来妊娠的角度来看，产前和植入前基因诊断是年轻父母可以考虑的选择。本文描述了儿童主要的肿瘤易感性。针对每种组织学亚型，结合这些主要肿瘤易感性讨论了不同的诊断方向。
