Objective: Spinal cord tumors constitute a small part of spinal surgery owing to their rarity. This retrospective study describes their current management.Methods: Forty-eight patients were treated for an intramedullary tumor between 2014 and 2020 at a single institution. Patients’ files were retrospectively studied. We detailed clinical status according to neurological deficit and ambulatory ability using the modified McCormick Scale, radiological features like number of levels, associated syringomyelia, surgical technique with or without intraoperative electrophysiological monitoring, pathological findings, and postoperative outcome.Results: The median age of this population was 43 years, including 5 patients under 18 years. The median delay before first neurosurgical contact was 3 months after the first clinical complaint. Treatment was gross total resection in 43.8%, subtotal resection in 50.0%, and biopsy in 6.2%. A laminectomy was performed for all the patients except 2 operated using the laminoplasty technique. Pathological findings were ependymoma in 43.8%, hemangioblastoma in 20.8%, and pilocytic astrocytoma in 10.4%. Six patients were reoperated for a tumor recurrence less than 2 years after the first surgical resection. One patient was reoperated for a postoperative cervical kyphosis.Conclusion: Intramedullary tumors are still a challenging disease and they are treated by various surgical techniques. They must be managed in a specialized center including a trained surgical, radiological, electrophysiological, and pathological team. Arthrodesis must be discussed before performing extensive laminectomy to avoid postoperative kyphosis.

Background: High-grade glioma (HGG), formerly known as Glioblastoma multiforme, can be complicated by hematomas, either at the initial presentation or after surgical removal. Rarely, postoperative bleeding can occur extra-axially, resulting in subdural hematomas (SDH) that might require surgical evacuation. Of note, little is known about the cellular composition of those hematomas. Observations: We present the case of a patient operated on for HGG who developed postoperative recurrent SDH that required multiple surgical evacuations. Histopathological analyses of the membranes comprising the SDH revealed the presence of HGG tumoral cells. Conclusions: Based on our observation, hematomas associated with HGG, either extra or intra-axial, should be suspected of being a sign of tumoral recurrence or spread and histopathological analyses might be considered as they could lead to further adaptation of the patient’s treatment.

Summary: First reported case of primary central nervous system infantile fibrosarcoma. Expansion of the molecular spectrum of infantile fibrosarcoma with identification of an RBPMS::NTRK3 fusion. Marked efficacy of larotrectinib observed in an unresectable CNS tumour. Reinforces the importance of molecular testing in atypical paediatric CNS tumours.

DNA methylation profiling of CNS tumors led to the identification of HPAP (high‐grade glioma with pleomorphic and pseudopapillary features), a recently proposed entity with variable morphology, recurrent MAP‐kinase pathway activating events, and longer survival compared to glioblastoma. We aimed to independently validate and further characterize this entity. We retrieved a multicentric cohort of gliomas compatible with HPAP and performed t‐SNE dimensionality reduction on their DNA methylation profile. Clinical, radiological, histological, and molecular data were reviewed. Twenty tumors clustering with previously reported HPAP cases were identified. Median age at diagnosis was 37 years. Radiologically, the tumors appeared as expansive lesions with heterogeneous enhancement and frequent cysts. Histologically, they were well‐circumscribed, with papillary and pleomorphic features variably present. Marked histological signs of aggressivity were present in seven. Immunostainings showed expression of GFAP, OLIG2, and CD34. All cases were IDH1/2 wildtype and pMGMT unmethylated. Recurrently mutated genes included TP53 , ATRX , RB1, and BRAF . Five patients had germline pathogenic variants in genes associated with hereditary tumor predisposition syndromes ( MLH1 , BRCA2 , CHEK2 , NF2 , RB1 ). All patients underwent surgical resection, but subsequent management was heterogeneous. Ten‐year estimated survival was 85%. CDKN2A homozygous deletion seemed to identify more aggressive tumors. In conclusion, our data suggest the existence of a novel entity of circumscribed gliomas with long‐term survival despite possible high‐grade histological presentation, for which we propose the nomenclature “Gliomas with pleomorphic and pseudopapillary features” with a spectrum encompassing provisory grade 2 and 3. A proper identification could guide treatment choices.