Background: Juvenile xanthogranuloma is one of the most common non-langerhan cell histiocytoses in children. Usually cutaneous, there are disseminated forms. However, neurological localizations remain exceptional.
Case Report: We report the case of a cerebellar localization, associated with cutaneous and pulmonary lesions in a 13-month old child. Surgical treatment of the cerebellar lesion was associated with vinblastin chemotherapy, that led to total regression of the cutaneous and pulmonary lesions, neurological stabilization, without recurrence on the control IRM, after a period of 7 years.
Discussion: This is the second reported case of histologically documented cerebellar juvenile xanthogranuloma. In the event of cutaneous juvenile xanthogranuloma, the neurological examination must be attentive and supplemented, in case of any suspicion, by medical imaging. We believe that surgical treatment of neurological juvenile xanthogranuloma is necessary, in the event of symptomatic lesions and accessible to surgery. No consensus has been reached on subsequent treatments (radiotherapy, chemotherapy) and must be discussed according to the clinical profile and data in the literature.
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Article Synopsis
- Juvenile xanthogranuloma (JXG) is a rare benign condition primarily affecting neonates and young children, often presenting as skin lesions, but intramuscular JXG is much less common, accounting for only 0.6% of cases.
- A case involving a 5-month-old girl showed a slow-growing lump in her left thigh, which was diagnosed as deep JXG after imaging, biopsy, and surgical resection.
- Histological findings revealed characteristics typical of JXG, and despite initial concerns about tumor margins, the patient has shown no signs of recurrence over 48 months post-surgery, highlighting the importance of proper diagnosis and monitoring.
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