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Juvenile xanthogranuloma with cutaneous and cerebral manifestations in a young infant
1Department of Laboratory Medicine, Shiga University of Medical Science, Japan.
Insights
Juvenile xanthogranuloma, typically a skin condition, can rarely affect the brain. This case highlights its potential for intracranial involvement, emphasizing the need for broader differential diagnoses.
Area of Science:
- Pathology
- Neurology
- Pediatrics
Background:
- Juvenile xanthogranuloma (JXG) is generally a benign, self-limiting skin condition.
- Intracranial involvement of JXG is exceptionally rare, posing diagnostic challenges.
Observation:
- A case report of an 8-month-old boy with a chest wall nodule and new-onset epileptic seizures.
- Surgical resection of both a subcutaneous chest tumor and a cerebral subcortical tumor in the left temporal lobe.
Findings:
- Histopathological examination revealed features consistent with JXG in both lesions, including characteristic cellular components.
- Immunohistochemical analysis supported a monohistiocytic origin, with tumor cells positive for lysozyme and CD68, but negative for S-100 protein.
Implications:
- This case underscores the importance of considering JXG in the differential diagnosis of pediatric intracranial histiocytic and xanthomatous lesions.
- Recognizing rare intracranial manifestations of JXG is crucial for appropriate patient management and treatment strategies.
Abstract:
Juvenile xanthogranuloma is usually a self-limiting disease of the skin. Intracranial manifestations are extremely rare. We report the clinico-pathological features of an 8-month-old boy suffering from a gradually enlarging nodule of the chest wall and subsequent epileptic seizures. The subcutaneous tumor and a cerebral subcortical tumor of the left temporal lobe were resected. The histological appearance of both tumors corresponded to juvenile xanthogranuloma and included histiocytes, foamy cells, giant cells, inflammatory cells, and collagen-producing fibroblasts showing a storiform pattern. Immunohistochemical studies demonstrated positivity of the tumor cells for lysozyme, CD68 and myeloid-histiocytic antigen, but not S-100 protein, supporting mono-histiocytic differentiation. This case indicates that juvenile xanthogranuloma should be considered in the differential diagnosis of intracranial "xanthomatous" and histiocytic lesions.