Department of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
Pediatr Blood Cancer. 2013 Feb;60(2):210-6. doi: 10.1002/pbc.24225. Epub 2012 Jun 12.
Medulloblastoma histological classification has gained in importance and newer treatment protocols will include histology stratification. We centrally reviewed medulloblastoma cases from past 10 years reassessing their histology to ascertain its prognostic significance.
Samples from 125 consecutive patients (99 males; 10 under age 3 years) were reviewed according to the two WHO classifications of 2000/2007.
Eighty-two patients did not have metastases, the primary tumor was completely resected in 101. The median follow-up was 96 months. Treatment was: our institutional protocol, that is, hyperfractionated accelerated radiotherapy (HART), for 39 non-metastatic cases up to 2003; according to the European PNET IV protocol in 31 cases; a HART-based strategy in 39 metastatic cases; tailored to the age below 3 years and based on high-dose chemotherapy in 10; and tailored to the patients conditions in 7. The 5-year PFS/OS rates were 76% and 81%, respectively. Histology was classic in 93 cases, nodular/desmoplastic in 20, anaplastic/large-cell in 9, and with extensive nodularity (MBEN) in 3. Stratification by residual disease after resection, metastases, age, or protocols was not prognostic. Histology suggested 5-year PFS rates of 82% for the desmoplastic and MBEN variants, 78% for classic medulloblastoma, 44% for the anaplastic/large-cell variants (P = 0.01). Multivariable analysis demonstrated statistically significant difference in PFS by histology (P = 0.02), due to the poor prognosis of anaplastic/large-cell medulloblastoma.
Tailoring treatments to known risk factors cancelled all prognostic differences, except for anaplasia (not considered as such within previous trials) which proved the most powerful prognostic factor, warranting appropriate treatment intensification.
髓母细胞瘤的组织学分类变得越来越重要，新的治疗方案将包括组织学分层。我们对过去 10 年的髓母细胞瘤病例进行了中心回顾，重新评估其组织学以确定其预后意义。
根据 2000/2007 年的两个世卫组织分类，对 125 例连续患者（99 名男性；10 名年龄在 3 岁以下）的样本进行了回顾。
82 例患者无转移，101 例患者的原发肿瘤完全切除。中位随访时间为 96 个月。治疗方法如下：我们机构的方案，即超分割加速放疗（HART），适用于 2003 年前的 39 例非转移性病例；根据欧洲 PNET IV 方案治疗 31 例；39 例转移性病例采用 HART 为基础的策略；10 例患者年龄在 3 岁以下，基于大剂量化疗；7 例患者根据病情而定。5 年无进展生存率/总生存率分别为 76%和 81%。组织学表现为经典型 93 例，结节/促纤维增生型 20 例，间变型/大细胞型 9 例，广泛结节型（MBEN）3 例。根据肿瘤切除后的残留疾病、转移、年龄或方案分层，无预后意义。组织学提示结节/促纤维增生型和 MBEN 变异型 5 年无进展生存率为 82%，经典型髓母细胞瘤为 78%，间变型/大细胞型为 44%（P=0.01）。多变量分析显示，组织学差异对无进展生存率有统计学意义（P=0.02），这是由于间变型/大细胞型髓母细胞瘤预后不良。
针对已知危险因素进行治疗，除间变（以前的试验中不认为是这样）外，所有预后差异都被消除，间变被证明是最有力的预后因素，需要适当加强治疗。
