Multiple intracranial juvenile xanthogranuloma not a straightforward diagnosis (a case report)

Abstract

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.

Juvenile Xanthogranuloma of the Head and Neck: Imaging Findings in 11 Cases

“…Dural-based and ventricular lesions are frequently associated with parenchymal involvement. 48 , 49 The high incidence of ventricular and meningeal lesions may be explained by the presence of macrophages and/or histiocytes in the choroid plexus and meninges. 38 In addition, the dura mater may be involved because of an aggressive, destructive bone JXG.…”

A rare case of agminated juvenile Xanthogranuloma: Clinical and dermatoscopic evolution

“…1 In rare cases, JXG can involve internal organs such as subcutaneous soft tissue, central nervous system, liver, spleen lung, eye, oropharynx and muscle. 4,5 The most common presentation is a well-demarcated, round to oval papule or nodule. Exceptional clinical features include hyperkeratotic and pedunculated lesions, the giant form (larger than 2.0 cm), 6 a generalized lichenoid eruption, a reticulated maculopapular eruption, a flat plaque-like lesion and a clustered form.…”