Jacques Grill, Cormac Owens
Brain Tumor Program, Department of Pediatric and Adolescent Oncology, Gustave Roussy Cancer Institute, Villejuif, France.
Handb Clin Neurol. 2013;112:931-58. doi: 10.1016/B978-0-444-52910-7.00015-5.
Central nervous system tumors are the most frequent malignant tumor in children and the main cause of death in this age group after traffic accidents. The current estimates are that one adult in 2500 is a survivor of a brain tumor that occurred during childhood. These tumors are particularly heterogeneous in terms of histology/biology, treatment, and outcome. They share, however, a high risk of neurological and cognitive morbidity due to the disease itself and the treatment modalities (radiotherapy, surgery, and chemotherapy). Diagnosis is frequently delayed because symptoms are usually nonspecific at the beginning of the evolution. Posterior fossa is the most frequent site and the tumors present most frequently with signs of intracranial hypertension. Supratentorial tumors are more frequent in infants and in adolescents; seizures are not uncommon, especially for benign tumors. When adjuvant treatment is needed, radiotherapy is usually the mainstay apart from some histologies where chemotherapy may be sufficient: low-grade gliomas, desmoplastic medulloblastomas, malignant glial tumors in infants. Multidisciplinary care is best performed in tertiary care centers and should include early rehabilitation programs soon after surgery.
中枢神经系统肿瘤是儿童中最常见的恶性肿瘤,是该年龄组继交通事故后主要的死亡原因。目前估计,每2500名成年人中有1人是童年期发生的脑肿瘤幸存者。这些肿瘤在组织学/生物学、治疗和预后方面特别具有异质性。然而,由于疾病本身和治疗方式(放疗、手术和化疗),它们都有很高的神经和认知发病风险。由于疾病进展初期症状通常不具特异性,诊断常常延迟。后颅窝是最常见的部位,肿瘤最常表现为颅内高压体征。幕上肿瘤在婴儿和青少年中更常见;癫痫发作并不少见,尤其是对于良性肿瘤。当需要辅助治疗时,除了某些组织学类型(如低级别胶质瘤、促纤维增生性髓母细胞瘤、婴儿恶性胶质肿瘤,化疗可能就足够)外,放疗通常是主要治疗手段。多学科护理最好在三级护理中心进行,并且应在手术后尽快纳入早期康复计划。