[Large nodular, multicenter, juvenile xanthogranuloma with ulceration]

Area of Science:

• Dermatology
• Pathology
• Pediatric Oncology

Background:

• Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic disorder typically affecting infants and young children.
• Multicentric JXG is an uncommon variant presenting with multiple lesions, posing diagnostic challenges.
• Accurate diagnosis is crucial for appropriate management and prognosis.

Observation:

• The patient presented with an unusual clinical and histological pattern that initially obscured the diagnosis of JXG.
• The tumor exhibited a spontaneous remission maintained for over two years.
• Histological examination revealed a transformation from a fibrocytic to a polymorphological histiocytic picture with numerous fat-storing giant cells.

Findings:

• The definitive diagnosis of multicentric juvenile xanthogranuloma was established based on the observed spontaneous remission and characteristic cellular changes.
• The absence of specific cell organelles in the lesional cells supported the diagnosis.
• The dynamic histological evolution was key to understanding the disease process.

Implications:

• This case highlights the importance of considering JXG even with atypical presentations.
• Understanding the natural history and histological variability of JXG is essential for accurate diagnosis.
• The findings contribute to the literature on rare pediatric histiocytic disorders and their management.