Madon E, Besenzon L, Brach del Prever A, Cordero di Montezemolo L, Cucchi B, Iannacci L, Mosso M L, Pession A, Paolucci G
Tumori. 1986 Jun 30;72(3):285-92. doi: 10.1177/030089168607200309.
This retrospective study was undertaken to evaluate the clinical characteristics, course and treatment of children (0-14 years of age) diagnosed with a primary CNS tumor during the period 1976-1982 in Italy. Four hundred and sixty-two patients (263 males and 199 females) were followed by 18 various neurosurgical and pediatric oncology centers. The histologic types most frequently reported were: medulloblastoma (23%), astrocytoma (16%), ependymoma (11%) and spongioblastoma (11%). Of the 388 patients who underwent surgery, radical excision was reported in 42%, partial excision in 32%, biopsy only in 6%, and unqualified surgery in 4%; 19% had no surgery. Radiotherapy and chemotherapy combined were administered in 61% of the 143 patients followed at pediatric oncology centers; 19% received radiotherapy alone, 3% chemotherapy alone, and 17% neither treatment. Forty-six percent of the patients were reported alive, 40% dead, and 14% lost to follow-up. Performance status was identified for 62 patients. The investigation revealed marked differences in the therapeutic treatment administered, thus precluding valid data analysis. This emphasizes the need to coordinate efforts among the institutions and the disciplines involved in the treatment of this form of childhood cancer.
本回顾性研究旨在评估1976年至1982年期间在意大利诊断为原发性中枢神经系统肿瘤的0至14岁儿童的临床特征、病程及治疗情况。18个不同的神经外科和儿科肿瘤中心对462例患者（263例男性和199例女性）进行了随访。最常报告的组织学类型为：髓母细胞瘤（23%）、星形细胞瘤（16%）、室管膜瘤（11%）和海绵状成细胞瘤（11%）。在接受手术的388例患者中，报告根治性切除的占42%，部分切除的占32%，仅活检的占6%，不合格手术的占4%；19%未接受手术。在儿科肿瘤中心随访的143例患者中，61%接受了放疗和化疗联合治疗；19%仅接受放疗，3%仅接受化疗，17%未接受任何治疗。报告46%的患者存活，40%死亡，14%失访。对62例患者进行了功能状态评估。调查显示，所给予的治疗存在显著差异，因此无法进行有效的数据分析。这强调了参与这种儿童癌症治疗的机构和学科之间需要协调努力。
