Leblond Pierre, Delebarre Mathilde, Aubert Sébastien
Centre Oscar-Lambret, unité d'oncologie pédiatrique, 3, rue F.-Combemale, 59020 Lille Cedex, France.
Bull Cancer. 2011 May;98(5):595-605. doi: 10.1684/bdc.2011.1357.
Adrenocortical carcinomas are rare in children and sometimes occur in patients with predisposing syndrome like Li-Fraumeni or Beckwith-Wiedemann syndromes. The diagnosis is often based on clinical, biological and radiological findings completed by histological examination. The gold standard of treatment remains radical surgery sometimes completed by a cytotoxic chemotherapy based on platinum-derived drugs, etoposide and doxorubicin, in association with mitotane in case of high risk of relapse. The role of radiation therapy is discussed because of the relatively frequent involvement of p53 mutations. The rarity of this type of disease and the poor prognosis of the locally advanced and metastatic forms must lead to the systematic registration of these patients and to the harmonization of the management in a national or even European level.
肾上腺皮质癌在儿童中罕见，有时发生于具有易感综合征（如李-弗劳梅尼综合征或贝克威思-维德曼综合征）的患者。诊断通常基于临床、生物学和放射学检查结果，并通过组织学检查加以完善。治疗的金标准仍然是根治性手术，对于复发风险高的患者，有时还需联合基于铂类药物、依托泊苷和阿霉素的细胞毒性化疗以及米托坦。由于p53突变相对常见，放疗的作用存在争议。这类疾病的罕见性以及局部晚期和转移性形式的预后不良，必须促使对这些患者进行系统登记，并在国家甚至欧洲层面统一管理。
