MB-80: OUTCOME OF CHILDREN WITH POSTERIOR FOSSA MEDULLOBLASTOMA

The purpose of the study was to evaluate outcome of patients with newly diagnosed medulloblastoma treated in Prague within 2000-2015. Demographics, treatment and survival data were collected in 94 consecutive patients (34 females, 60 males) with histologically proven medulloblastoma. Two patients were lost to follow up. Median age at the time of diagnosis was 8.43 (0.29-17.6) years. Kaplan-Meier analysis was performed to determine survival. Two out of 16 infants (5 metastatic) died before the start of any therapy and 14 were treated with chemotherapy only. Older patients (n = 75) were treated with radiotherapy and chemotherapy, 44 as standard risk (SR) and 31 as high risk (HR) group (14 metastatic). One patient with biallelic BRCA2/FANCD1 mutation underwent individualized treatment. The 5-year PFS and OS of all 92 analysed patients were 66.8% and 72.3%. Survival was higher in older children treated with radiotherapy with 5-year PFS and OS 75.9% and 76.9% respectively. 5-year OS of patients with SR group was 83.0% and HR group 67.9%. The 5-year OS of treated infant medulloblastoma patients was 62.5%. Three out of six relapsed patients were salvaged with radiotherapy. Strikingly, the incidence of secondary malignancies (SNM) reached 9% (n = 9 in 8 patients) and SNM were responsible for 4 deaths (GBM (n = 2), sarcoma (n = 1) and MDS (n = 1). The prognosis of medulloblastoma patients is generally good with over 70% survival with current therapies. Incidence of SNM is worrisome and contributes to increased morbidity and mortality. Supported by the “Project for Conceptual Development of Research Organization 00064203”