Purpose Young patients with a brain tumour show neurocognitive alterations as both consequences of the tumour and of the treatments received. In this paper, we present the basal analysis of a prospective study of damage from radiation after focal radiation therapy (RT), correlating tumour localization, symptoms, neurological/endocrinological impairments, surgery/ies±chemotherapy, and cognitive assessments at the time of enrolment. Methods Sixty-six children eligible for focal RT underwent a neurocognitive assessment. The demographic, pathological and clinical variables with MRI morphological scans, where differenypt kind of damage scores were defined, were then analysed. Results The patients’ median age was 8 years; the most frequent tumour was ependymoma (41%), and the posterior fossa (29%) was the prevalent site. All but 2 children (with germ cell tumours), had undergone surgery and 32 sessions of chemotherapy before irradiation. Ad-hoc scores for neurological deficits and endocre alterations were created and structural abnormalities were scored in each cortical/subcortical region. Patients with posterior fossa ependymomas and infratentorial tumours showed the highest score of neurological damage while endocrine alterations were more serious in patients with craniopharyngioma and germ cell tumours of the sellar region and ventricular system. The median number of structural damaged areas was equal to 2 for each child. Neurological deficit scores were not associated with the presence of hydrocephalus and surgery/ies received, unlike endocrine deficits. Conclusion The analysis of baseline evaluations highlights damage existing prior to radiation and generated by multiple factors. In light of these findings, damage over time should be investigated by distinguishing multiple generating factors.

Introduction. The H3K27M-mutant diffuse midline glioma (DMG) was first included in the WHO Classification of CNS (central nervous system) tumors in 2016, and confirmed in its fifth edition. The biological behavior and dismal prognosis of this tumor resemble DIPG (diffuse intrinsic pontine gliomas). Homogeneously-treated series are rarely reported. Methods. From 2016 onwards, we treated patients with DMG with radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus re-irradiation at relapse, as already done for DIPG (DOI10.1007/s11060-014-1428-z). Results. We treated nine patients, seven females, median age at diagnosis of 13 years-old. Tumor sites were: thalamic in five cases, pontocerebellar in two, pineal in one, and paratrigonal with nodular/leptomeningeal dissemination in one. Three patients were biopsied, and six had partial tumor resections. Central review of the pathologists’ diagnoses was performed. The median time to local progression was 12.7 months, and the median overall survival was 17.8 months. Six patients died of tumor progression, one of cerebral bleeding whose tumor was progressing. Two were alive, one in continuous remission, the other after a relapse, at 38.6 and 46.3 months after diagnosis, respectively. Progression-free survival was 33.3% at one year. Overall survival was 88.9%, 33.3% and 22.2% at 1, 2 and 3 years, respectively. Conclusions. This is one of only a handful of reports on homogeneously-treated series. The results obtained are comparable with those seen in patients with DIPG. Given the phenotypically- and molecularly-defined setting of DMG and severe outcome in this orphan population, they should be treated and included in registries and protocols of DIPG.