Introduction and importance Juvenile xanthogranuloma (JXG) rarely presents as multifocal intracranial disease in the paediatric population. Therefore, this case of extensive tumour burden, primarily within the lateral ventricles, presented a neurosurgical challenge on numerous fronts. Presentation of case This is the case of a 9-year-old male presenting with a 2-year history of visual disturbances. Radiographic imaging demonstrated extensive intracranial masses involving both lateral ventricles, the straight sinus and right cerebellum. A staged tumour resection was planned, targeting the lesions within the right lateral ventricle initially. Complete resection was achieved during surgery. Post-operative morbidity showed a decline in the patient's functional status with respect to mobility and communication, Glasgow outcome scale 3. Extensive immunohistochemical analysis ultimately revealed a diagnosis of JXG. The patient is undergoing chemotherapy, with subsequent surgical resection being dependent on overall recovery. Clinical discussion JXG is the most common form of non-Langerhans histiocytosis and typically arises as a cutaneous disorder during early childhood. It is a rare cause of extensive intracranial tumour burden, with limited publications of this kind in the literature. This is even more atypical given the absence of any of the classic cutaneous morphology seen in JXG. Conclusion JXG involving the central nervous system is a rare encounter. Therefore, a clear algorithm for the management of a case of extensive intracranial tumours resulting from JXG has not been defined. This only amplifies the difficulty in treating these cases.
Intraventricular schwannomas are rarely encountered in neurosurgical practice. The development and progression of a schwannoma within the ventricular system is still a hypothesised theory. Here, we present a case of a 59-year-old female who presented with a three-week history of headaches. Her symptoms progressively worsened, with resultant altered mental status within the last week. Imaging scans of the brain demonstrated a well-defined mass within the right lateral ventricle with associated midline shift and obstructive hydrocephalus. A parietal craniotomy and resection of the intraventricular mass was performed. Her postoperative course was uneventful. Histopathological assessment depicted a biphasic pattern of Antoni A and B, with a strongly positive S100. This was in keeping with an intraventricular schwannoma. The diagnosis of an intraventricular schwannoma does not fit within the classical differential framework for ventricular masses. These tumours are extremely uncommon but fortunately, they are typically benign. Therefore, adequate surgical resection remains the gold standard of care for these unfamiliar masses.
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