Juvenile Xanthogranuloma: Presenting as an Isolated Renal Involvement

Area of Science:

• Pediatric Histiocytic Disorders
• Pediatric Nephrology
• Pediatric Oncology

Background:

• Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children.
• While commonly presenting with skin lesions, extracutaneous and visceral involvement are documented.
• Renal JXG is infrequently reported, usually alongside cutaneous manifestations.

Observation:

• This case presents an unusual instance of isolated renal JXG.
• The JXG manifested as a renal mass with adjacent soft tissue and muscle involvement.
• Crucially, the patient exhibited no cutaneous lesions or other signs of JXG on the skin.

Findings:

• This represents the first documented case of JXG solely involving the kidney without any skin manifestations.
• The isolated renal presentation highlights the diverse clinical spectrum of JXG.
• Diagnostic imaging and subsequent histopathology confirmed the diagnosis of renal JXG.

Implications:

• This case expands the understanding of JXG's potential presentations.
• It underscores the importance of considering JXG in pediatric renal masses, even without skin findings.
• Further research may elucidate the mechanisms behind isolated extracutaneous JXG.