Department of Medical Oncology and Hematology, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian 1, 20133, Milan, Italy.
SOSD Oncologia Integrata del Giovane e Radioterapia Pediatrica, Centro di Riferimento Oncologico (CRO) IRCCS, Aviano, Italy.
Cancer Metastasis Rev. 2019 Dec;38(4):683-694. doi: 10.1007/s10555-019-09831-3.
Wilms tumor (or nephroblastoma), rhabdomyosarcoma, and medulloblastoma, common embryonal tumors in children, can occasionally occur in adults, for whom survival is significantly inferior than pediatric patients. Available data on adults with Wilms tumor consist of case or case series reports. Among other factors, the unfamiliarity of adult oncologists and pathologists with nephroblastoma and consequent delays in initiating the appropriate risk-adapted chemotherapy may negatively influence outcomes. The survival decrement in adults with rhabdomyosarcoma has been attributed to the lack of centralized care, the inconsistent use of standard protocol-driven multimodal therapy, and lower chemotherapy tolerance in adult patients. In children with medulloblastoma, evidence from randomized clinical trials has led to risk-tailored therapies tuned on histology, extent of initial disease, and biological features. Such refinements are still missing for adults due to the lack of similar trials and studies that might provide the same or a different understanding regarding patients' individual prognosis, treatment morbidity, and quality of life. Recent experiences have suggested that applying or adjusting pediatric protocols to adult patients with these tumors is feasible and can improve survival. Here, we provide an evaluation of the current evidence for the management of Wilms tumor, rhabdomyosarcoma, and medulloblastoma arising in adults. This review aims to promote the referral of adolescents and adults with pediatric tumors to pediatric centers for inclusion into pediatric protocols, or into protocols and studies specifically designed for that age group with the cooperation between pediatric and adult oncologists.
Wilms 肿瘤(或肾母细胞瘤)、横纹肌肉瘤和髓母细胞瘤是儿童常见的胚胎性肿瘤,偶尔也会发生在成人中,成人的生存率明显低于儿童患者。关于成人 Wilms 肿瘤的可用数据包括病例或病例系列报告。除其他因素外,成人肿瘤学家和病理学家对肾母细胞瘤不熟悉,导致延迟开始适当的风险适应化疗,这可能对结果产生负面影响。成人横纹肌肉瘤生存率下降归因于缺乏集中治疗、标准方案驱动的多模式治疗使用不一致以及成人患者对化疗的耐受性降低。对于患有髓母细胞瘤的儿童,来自随机临床试验的证据导致了针对组织学、初始疾病程度和生物学特征的风险定制治疗。由于缺乏类似的试验和研究,这些研究可能对患者的个体预后、治疗发病率和生活质量有相同或不同的理解,因此对于成人来说,仍然缺少这些细化。最近的经验表明,将儿科方案应用于或调整为患有这些肿瘤的成年患者是可行的,并可以提高生存率。在这里,我们对成人中发生的 Wilms 肿瘤、横纹肌肉瘤和髓母细胞瘤的管理的现有证据进行了评估。这篇综述旨在促进将患有儿科肿瘤的青少年和成年人转介到儿科中心,以便纳入儿科方案,或与儿科和成人肿瘤学家合作,纳入专为该年龄组设计的方案和研究。