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Simple Summary: Central Nervous System (CNS) pediatric tumors represent the most common solid tumors in children with a wide variability in terms of survival and therapeutic response. Unlike their adult counterparts, the mutational landscape of pediatric CNS tumors is mostly characterized by oncogenic fusions rather than multiple mutated genes. We report four pediatric cases associated with rare oncogenic fusions, providing an overview of oncogenic fusion pathogenesis, histological phenotype, diagnostic and theranostic impact. Our work underlines that most of these rare oncogenic fusions are not specific to a single morpho-molecular entity among the pediatric CNS tumors. Even within tumors harboring the same oncogenic fusions, a wide range of morphological, molecular and epigenetic entities can be observed. These findings highlight the need for caution when applying the fifth CNS WHO classification, as the vast majority of these fusions are not yet incorporated in the diagnosis, including grade evaluation and DNA methylation classification. Background and Objectives: Central Nervous System (CNS) pediatric tumors represent the most common solid tumors in children with a wide variability in terms of survival and therapeutic response. By contrast to their adult counterpart, the mutational landscape of pediatric CNS tumors is characterized by oncogenic fusions rather than multiple mutated genes. CNS pediatric tumors associated with oncogenic fusions represent a complex landscape of tumors with wide radiological, morphological and clinical heterogeneity. In the fifth CNS WHO classification, there are few pediatric CNS tumors for which diagnosis is based on a single oncogenic fusion. This work aims to provide an overview of the impact of rare oncogenic fusions (NTRK, ROS, ALK, MET, FGFR, RAF, MN1, BCOR and CIC genes) on pathogenesis, histological phenotype, diagnostics and theranostics in pediatric CNS tumors. We report four cases of pediatric CNS tumors associated with NTRK (n = 2), ROS (n = 1) and FGFR3 (n = 1) oncogenic fusion genes as a proof of concept. Cases presentation and literature review: The literature review and the cohort that we described here underline that most of these rare oncogenic fusions are not specific to a single morpho-molecular entity. Even within tumors harboring the same oncogenic fusions, a wide range of morphological, molecular and epigenetic entities can be observed. Conclusions: These findings highlight the need for caution when applying the fifth CNS WHO classification, as the vast majority of these fusions are not yet incorporated in the diagnosis, including grade evaluation and DNA methylation classification. [ABSTRACT FROM AUTHOR]

Introduction: Craniopharyngiomas (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. Methods: We 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. Results: Nineteen 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. Conclusion: CP 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. [ABSTRACT FROM AUTHOR]

External hydrocephalus (EH) is a recognised sub-type of hydrocephalus associated with macrocephaly in infancy. EH is characterised by the enlargement of subarachnoid spaces (so-called subarachnomegaly) with a normal ventricular system on brain imaging. EH is traditionally considered benign and self-limiting, yet its pathophysiology remains puzzling. Mounting evidence for an association between EH and hydrovenous disorders reshapes our understanding of this condition and its management. To our knowledge, we report the first association between EH and dural arteriovenous fistula (dAVF) in a 17-months-old boy. As dAVF may be a life-threatening condition, early diagnosis and optimal treatment are critical. This case epitomises the intricacies of EH's aetiology and associated conditions requiring careful management. Therefore, we recommend considering MR angiography in EH's workup and long-term follow-up. Our experience supports the ongoing reconsideration of EH's presumed benignity. [ABSTRACT FROM AUTHOR]

To determine the possible relation between cervical sagittal balance and neck pain in patients having anterior cervical spine (ACS) surgery. Retrospective monocentric study on 85 patients who underwent ACS surgery between 2014 and 2016. Clinically, all patients were assessed using the Neck Disability Index (NDI). Radiological assessment was achieved by MRI or CT-scan of the cervical spine allowing measurement of radiological parameters for preoperative cervical sagittal balance. These same criteria were measured postoperatively using X-rays. There is a statistically significant increase in the Cobb angle postoperatively (10.34 degrees) compared to preoperatively (6.68 degrees) (p < 0.05). Concomitantly, there is a statistically significant decrease in NDI postoperatively (22.69%) compared to preoperatively (42.31%) (p < 0.01). There is a negative correlation between Cobb angle and NDI (r= −0.31) (p < 0.05). An improvement in the cervical sagittal balance after ACS surgery is accompanied by a reduction of neck pain. Radiological parameters of cervical sagittal balance may be taken into account when planning surgery in order to maintain cervical alignment and thereby limit the occurrence of neck pain. [ABSTRACT FROM AUTHOR]

Aims: Goal of Transvenous Lead Extraction (TLE) is complete removal of all targeted leads, without complications. Despite counter traction manoeuvres, efficacy rates are often hampered by broken right ventricle lead (RV‐lead) tips. Mechanically powered lead extraction (Evolution sheath) is effective, however safety of dissection up to the lead tip is unclear. Therefore, we examined the feasibility and safety of RV‐lead extraction requiring dissection up to the myocardium. Methods and results: From 2009 to 2018, all TLE in the Isala Heart Centre (Zwolle, The Netherlands) requiring the hand‐powered mechanical Evolution system to extract RV‐leads (n = 185) were examined from a prospective registry. We assessed 4 groups: TLE with the first generation Evolution (n = 43) with (A1,n = 18) and without (A2,n = 25) adhesions up to the myocardium and TLE with the Novel R/L type (n = 142) of sheath with (B1, n = 59) and without (B2, n = 83) adhesions up to the myocardium. Complete success rate in Group B was significantly higher than group A (96.5 vs 76.7%, p = 0.0354). When comparing the patients with adhesions up to the myocardium, total complete success is higher in the R/L group (61.1% vs 90.5%, p = 0.0067). There were no deaths. Overall major complication rates were low (2/185; 1.1%) and there was no statistically significant difference in major and minor complications between the two groups. Conclusion: Extraction strategy with the bidirectional Evolution R/L sheath for right ventricular leads with adhesions up to the myocardium is safe and feasible. [ABSTRACT FROM AUTHOR]