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IntroductionCraniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients.MethodsWe performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles.ResultsNineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery.ConclusionCP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.

Introduction: Technical advances and the increasing role of interdisciplinary decision-making may warrant formal definitions of expertise in surgical neuro-oncology. Research question: The EANS Neuro-oncology Section felt that a survey detailing the European neurosurgical perspective on the concept of expertise in surgical neuro-oncology might be helpful. Material and methods: The EANS Neuro-oncology Section panel developed an online survey asking questions regarding criteria for expertise in neuro-oncological surgery and sent it to all individual EANS members. Results: Our questionnaire was completed by 251 respondents (consultants: 80.1%) from 42 countries. 67.7% would accept a lifetime caseload of >200 cases and 86.7% an annual caseload of >50 as evidence of neuro-oncological surgical expertise. A majority felt that surgeons who do not treat children (56.2%), do not have experience with spinal fusion (78.1%) or peripheral nerve tumors (71.7%) may still be considered experts. Majorities believed that expertise requires the use of skull-base approaches (85.8%), intraoperative monitoring (83.4%), awake craniotomies (77.3%), and neuro-endoscopy (75.5%) as well as continuing education of at least 1/year (100.0%), a research background (80.0%) and teaching activities (78.7%), and formal interdisciplinary collaborations (e.g., tumor board: 93.0%). Academic vs. non-academic affiliation, career position, years of neurosurgical experience, country of practice, and primary clinical interest had a minor influence on the respondents’ opinions. Discussion and conclusion: Opinions among neurosurgeons regarding the characteristics and features of expertise in neuro-oncology vary surprisingly little. Large majorities favoring certain thresholds and qualitative criteria suggest a consensus definition might be possible.

Introduction: The incidence of intramedullary spinal cord tumors ranges from 2 to 4% of all central nervous system tumors. Only 6–8% are astrocytomas. The gold standard to diagnose a spinal cord tumor is the spinal cord MRI in toto. Specific radiological criteria orient the diagnosis of the intradural intramedullary lesion. Most of the authors studied adult populations of astrocytomas. However, pediatric astrocytomas present certain particularities. Research question: This work aims to determine if the usual radiological criteria of intramedullary astrocytomas (IMAs) are different depending on the patient's age. Material & methods: We evaluated the radiological features of IMAs in adult and pediatric groups through a retrospective study. Results: We collected 31 patients with IMAs (11 children and 20 adults). We observed some trends but we did not highlight any statistically significant difference between all the radiological criteria studied (sagittal and axial spinal cord localization, T1-and T2-weighted characteristics, contrast uptake, infiltrating character, presence of necrosis, heterogeneous lesion, necrotic, hemorrhagic, presence of edema) and the patient's age. Discussion & conclusion: Given the rarity of IMAs and the lack of large specific pediatric studies, it seems essential to routinely report all cases encountered and create multicentric pediatric databases to draw more robust conclusions.

Abstract Central Nervous System (CNS) embryonal tumors represent a heterogeneous group of highly aggressive tumors occurring preferentially in children but also described in adolescents and adults. In 2021, the CNS World Health Organization (WHO) classification drastically changed the diagnosis of the other CNS embryonal tumors including new histo-molecular tumor types. Here, we report a pediatric case of a novel tumor type among the other CNS embryonal tumors classified within the methylation class “CNS Embryonal Tumor with BRD4–LEUTX Fusion”. The patient was a 4-year girl with no previous history of disease. For a few weeks, she suffered from headaches, vomiting and mild fever associated with increasing asthenia and loss of weight leading to a global deterioration of health. MRI brain examination revealed a large, grossly well-circumscribed tumoral mass lesion located in the left parietal lobe, contralateral hydrocephalus and midline shift. Microscopic examination showed a highly cellular tumor with a polymorphic aspect. The majority of the tumor harbored neuroectodermal features composed of small cells with scant cytoplasm and hyperchromatic nuclei associated with small “medulloblastoma-like” cells characterized by syncytial arrangement and focally a streaming pattern. Tumor cells were diffusely positive for Synaptophysin, CD56, INI1 and SMARCA4 associated with negativity for GFAP, OLIG-2, EMA, BCOR, LIN28A and MIC-2. Additional IHC features included p53 protein expression in more than 10% of the tumor’s cells and very interestingly, loss of H3K27me3 expression. The Heidelberg DNA-methylation classifier classified this case as “CNS Embryonal Tumor with BRD4:LEUTX Fusion”. RNA-sequencing analyses confirmed the BRD4 (exon 13)–LEUTX (exon 2) fusion with no other molecular alterations found by DNA sequencing. Our case report confirmed that a new subgroup of CNS embryonal tumor with high aggressive potential, loss of H3K27me3 protein expression, BRDA4–LEUTX fusion, named “Embryonal CNS tumor with BRD4–LEUTX fusion”, has to be considered into the new CNS WHO classification.

Introduction: We report a case of a Kernohan notch phenomenon caused by an acute life-threatening intracranial subdural hematoma due to a cerebrospinal fluid (CSF) leak following lumbar surgery. Case report: A 71-year-old patient underwent decompressive lumbar surgery for refractory neurogenic claudication. Intraoperatively, a dural tear was noted and repaired. Two days later, the patient presented a sudden loss of consciousness following a CSF outflow in the subfascial lumbar drain. A head CT revealed an acute intracranial subdural hematoma with mass effect. A craniotomy was performed to evacuate the hematoma. The lumbar operative site was inspected and revealed a CSF leakage due to a dural defect which was subsequently repaired. The patient subsequently developed a Kernohan's notch phenomenon which partially recovered after two months of rehabilitation. Conclusion: We report a rare and dramatic case of Kernohan notch phenomenon due to an acute intracranial subdural hematoma occurring in the setting of a CSF leak following lumbar spine surgery. This case demonstrates the need to be aware of this potentially fatal complication after spine surgery and the importance of early diagnosis and appropriate treatment.

Background: Neuroendocrine dysfunction is a common complication of several neurosurgical conditions. In particular, central diabetes insipidus (CDI) can occur subsequent to traumatic brain injury, subarachnoid hemorrhage, cerebral tumors or as a result of a complication following pituitary neurosurgery. In contrast, surgical resection of non-sellar tumors does not commonly result in CDI, with only a few cases reported in the literature. Case description: We report the case of a 40-year-old man who presented a transient CDI following surgical resection of a pineal papillary tumor via an occipital interhemispheric trans-tentorial approach. The underlying pathogenesis of CDI occurring post resection of tumors arising at a distance from the sella is not yet clearly understood, especially since there is no evidence of direct compression of the pituitary stalk. With regards to our case, we hypothesize that restauration of the initial obstructive hydrocephalus might induce a rapid intracranial pressure variation leading to hemodynamics changes of the portal hypophyseal vascular system. Postoperative air entrapment in the sellar region might also lead to irritation of the pituitary stalk. Conclusion: This case highlights that CDI may happen as a consequence of pineal tumor resection and advocates for a careful postoperative monitoring.

Cluster headaches are characterized by painful unilateral periorbital attacks accompanied by dysautonomic symptoms. Although cluster headaches are classified as a primary headache disorder, a few cases of inflammatory, vascular or neoplastic conditions have been reported to cause or mimic cluster headaches. This raises the question as to the possible causal relationship between symptoms and an underlying structural lesion that may potentially interfere with the trigemino-autonomic reflex.We hereby report the first pediatric case of a brainstem cavernoma causing cluster headache-like symptoms. Of note, the child experienced pain relief after surgery.With regards to the pathophysiology, the observed ptosis observed in our child during the painful crises might be explained by mass effect of the cavernorma on the cisternal portion of the third cranial nerve. In addition, chronic inflammation of mesencephalic and motor nuclei of the trigeminal nerve by thalamo-pedoncular edema surrounding the lesion may have participated to painful pathways.This case illustrates that cluster headaches may occasionally be secondary to an underlying lesion. This advocates the need for MRI screening, especially as neurosurgical intervention may offer significant symptomatic relief and an improvement in the quality of life of these patients.