Introduction

Medulloblastoma (MB) is the most common primary malignant intracranial tumor in childhood, but it is very rare in adults, and for this reason, the optimal treatment has not yet been defined. We designed a multicentric study in order to define relevant outcome measures for future prospective studies.

Materials and methods

The project involved 10 Italian centers. The database shared among the centers contains epidemiological, diagnostic (radiological and histological/molecular), therapeutic, recurrence information, and survival data.

Results

A total of 152 patients (102 males and 50 females, median age 32) were included in the study. Twenty-three of 152 patients had a diagnosis of classic medulloblastoma, 52/152 had desmoplastic/extensive nodularity, 2/152 had large-cell anaplastic medulloblastoma, and the remaining had diagnoses not otherwise specified. Almost all patients underwent craniospinal irradiation after surgery; in 85.5% of patients, adjuvant chemotherapy, mainly platinum- and etoposide-based chemotherapy, was performed immediately after RT. Upon recurrence, most patients were retreated with various chemotherapy regimens, including intrathecal chemotherapy in patients with leptomeningeal dissemination. The overall survival (OS) rate at 5 years was 73.3% (95% CI, 65.0–80.0%). The median OS for the whole group of patients was 112 months.

Conclusions

The data collected were mainly consistent with the literature. A limitation of this study was the large number of patients lost to follow-up and the lack of molecular data for most patients diagnosed until 2010. An important challenge for the future will be MB biologic characterization in adults, with the identification of specific genetic patterns. It will be important to have more national and international collaborations to provide evidence-based management strategies that attempt to obtain a standard of care.

中文翻译：

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成人髓母细胞瘤：来自AINO（意大利神经肿瘤学会）和SIN（意大利神经病学会）的多中心回顾性研究的结果。

介绍

髓母细胞瘤（MB）是儿童时期最常见的原发性颅内恶性肿瘤，但在成年人中非常罕见，因此，目前尚无最佳的治疗方法。我们设计了一项多中心研究，以便为未来的前瞻性研究定义相关的结果指标。

材料和方法

该项目涉及10个意大利中心。各中心之间共享的数据库包含流行病学，诊断（放射学和组织学/分子），治疗，复发信息和生存数据。

结果

该研究共纳入152例患者（男102例，女50例，中位年龄32岁）。152例患者中有23例诊断为经典的髓母细胞瘤，52/152例为增生/广泛结节，2/152例为大细胞间变性髓母细胞瘤，其余诊断未另作说明。手术后几乎所有患者都接受了颅骨放射线照射；在85.5％的患者中，RT后立即进行辅助化疗，主要是基于铂和依托泊苷的化疗。复发后，大多数患者都接受了各种化学疗法的治疗，包括对有脑膜脑膜播散的患者进行鞘内化疗。5年总生存率为73.3％（95％CI，65.0-80.0％）。整个患者组的中位OS为112个月。

结论

收集的数据主要与文献一致。这项研究的局限性在于，有大量患者失去随访，并且大多数患者直到2010年都被诊断出缺乏分子数据。未来的一个重要挑战将是成人的MB生物学表征以及特定遗传模式的鉴定。 。重要的是要有更多的国家和国际合作，以提供试图获得护理标准的循证管理策略。