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BACKGROUND/OBJECTIVES: Low-grade-gliomas(LGGs) encompass a heterogeneous set of tumors of different histologies. They are the most common type of pediatric brain tumors accounting almost 30-50% of them. This is the first attempt to describe characteristics of Greek-LGG-patients diagnosed in the largest Pediatric Brain Tumors National Center. DESIGN-METHODS: We performed a retrospective review of newly-diagnosed LGG-patients during 2015-2017. Gender, age, symptoms, location, leptomeningeal infiltration, histological type, BRAF-status, NF-status, management and outcome were analysed. RESULTS: During the study period, 42 LGG-patients(22males) were diagnosed. Median age was 6.3years(range:40days-15.8years). Nearly 80% of the patients had symptoms of raised intracranial-pressure (headache/vomiting). The most common locations were: posterior-fossa(38.0%) followed by optic-pathway(26.2%). Surgical intervention was possible in 30 patients(26 subtotal/total excision, 2 partial excision, 2 biopsy). Leptomeningeal infiltration at diagnosis was detected in 2 patients. Histological type was mostly astrocytoma (23pts, 54.8%), especially pilocytic type (69.5% of astrocytomas). NF1 was diagnosed in 23.8% of the patients. In 16non-NF1 patients RT-PCR was performed for KIAA1549/BRAF-fusion and was positive in 5 patients and BRAFV600Emutation was detected in 3 patients. Treatment according to LGG2004-protocol was administered in 13 patients, of which 5 needed treatment-modification (3 due to carboplatin allergic reaction and 2 due to progressive disease) Radiotherapy was administered in 2 patients. One patient had a malignant transformation to glioblastoma multiforme, 16 months after initial diagnosis and died. At the end of treatment, visual and mobility problems were noted in two and one patients respectively. CONCLUSIONS: The most common histologic type was pilocytic astrocytoma. Overall survival was 97,6%. Our findings agree with those of internationally published studies.

CNS tumors are the second most common neoplasms in children and primary cause of childhood cancer morbidity. With the increase of 5-year survival rate, the diagnosis of long-term consequences is necessary. Recent studies suggest that childhood CNS tumors are associated with IQ decline and deficits in specific cognitive functions. Factors such as localization, age at diagnosis and therapeutic modalities influence the range and severity of the neuropsychological deficits. OBJECTIVE: This work presents the results of the first systematic evaluation of neuropsychological performance of Greek children with CNS tumors. METHODS: 72 children underwent neuropsychological testing between September 2015 and December 2017. Forty five children (29 boys, 64%) with mean age of 10.5 (range: 5.3-16.1) years were included in the study. Eighteen (40%) patients had low grade tumors. All patients underwent a comprehensive neuropsychological assessment, covering all domains of cognitive functioning. Impairment was defined as a performance of one standard deviation below the normative mean. RESULTS: Poor neurocognitive performance was found regarding attention (-2sd), speed of processing (-1.5sd), verbal memory (-2sd), learning (-1.5sd) and executive functions (-2sd). Despite the abovementioned deficits, intellectual efficiency was relatively preserved and fell within low average levels (>16%ile). CONCLUSIONS: Our results are in line with previous reports suggesting that children who have been treated for CNS tumors present with a wide range of long-term effects on cognitive functioning. Longer follow-up and expansion of the sample may give for further insights on the factors associated with poor neurocognitive outcome and allow appropriate intervention for optimal outcome.