P11.12.A Primary central nervous system neuroblastoma and ganglioneuroblastoma in adult patients. Clinical and molecular features

Abstract Background The clinical, morphological, molecular features, treatment modalities, and survival of primary central nervous system neuroblastoma ganglioneuroblastoma (CNS-NB CNS-GNB) in adult patients are still poorly understood. Material Methods study included 15 with CNS-NB 16 CNS-GNB aged 18 years older who were treated our clinic from 2008 to 2020. Clinical, radiological data surgical results analyzed. Analysis tumor profile was performed using the gene panel, which contained majority clinically significant genes. Results Progression-free (PFS) overall (OS) higher than CNS-GNB. Median PFS 156 46 weeks, respectively (p=0.022), median OS 541 82 (p=0.00045). In both groups, disease progression more favorable for localization cerebral hemispheres without involvement basal structures (p <0.05). Differences obtained when volume removed by 50% a weeks (p=0.042) compared biopsy. CNS-GNB, total resection increased subtotal (p=0.014 p=0.017), respectively. CNS-NB, no benefit observed any different first-line chemotherapy regimens > 0.05). group 6 cycles temozolomide other chemotherapeutic (p=0.026). high level expression only βIII-tubulin (54%, 7/13). levels found three genes: PDGFR-α 7/13), VEGF (85%, 11/13). No statistically effect mutation IDH1(R132H) on observed. influenced low or medium (p=0.006), ERCC1 (p=0.03), MGMT (p=0.029) (p=0.002). Conclusion morphologic type determined survival. localization. most important prognostic marker VEGF, while predictive markers.