Spinale Raumforderung – Astrozytom vs. diffuses Mittelliniengliom

Due to the new WHO Classification 2021, primary brain tumors are classified based not only on histology but also on molecular genetic testing. We report a case of a 30-year-old man presenting with persistent severe back pain in the thoracic spine and tingling paresthesia in both legs. Magnetic resonance imaging (MRI) of the spine showed an intramedullary tumor in the region of Th8/Th9. After case presentation and discussion at the neuro-oncological tumor board, tumor extirpation was performed. Histologically, a pleomorphic glioma with loss of ATRX expression and negative IDH mutation DD: astrocytoma (CNS grade 3) was considered. Integration of neuromolecularpathology resulted in the diagnosis of a H3 K27-altered diffuse midline glioma, grade 4. The patient received radiotherapy with concomitant and adjuvant chemotherapy. MRI follow-ups every 3 months showed no sign of recurrence. The H3 K27-altered diffuse midline glioma is a rare, highly aggressive brain tumor and occurs mostly in the brain stem or thalamus. The prognosis of H3 K27-altered diffuse midline glioma remains poor; the recommended first-line therapy is radiotherapy.