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Multifocal pigmented villonodular synovitis in a child.

J P Walls, J Nogi

    Journal of Pediatric Orthopedics
    |March 1, 1985
    PubMed
    Summary
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    A rare case of multifocal pigmented villonodular synovitis in a 6-year-old girl is presented. This youngest patient documented with multifocal disease highlights the varied presentation of this rare joint disorder.

    Area of Science:

    • Pediatric Orthopedics
    • Musculoskeletal Oncology
    • Pediatric Rheumatology

    Background:

    • Pigmented villonodular synovitis (PVNS) is a rare, benign neoplastic proliferation of the synovium.
    • Multifocal PVNS is exceptionally rare, particularly in pediatric populations.
    • Understanding the epidemiology and clinical presentation in children is crucial for early diagnosis.

    Observation:

    • A 6-year-old female presented with multifocal pigmented villonodular synovitis.
    • Lesions involved the right knee and the synovium of the posterior tibial tendons near the medial malleoli of both ankles.
    • This represents the youngest reported case of multifocal PVNS and the sixth pediatric case overall.

    Findings:

    • The patient exhibited a rare, disseminated form of PVNS.

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  • The involvement of multiple joints and tendon sheaths underscores the potential for widespread disease.
  • Diagnostic challenges in pediatric multifocal PVNS are highlighted by this case.

    • Implications:

    • This case expands the known spectrum of PVNS in children.
    • It emphasizes the need for thorough evaluation in pediatric patients with suspected joint or tendon sheath masses.
    • Further research into the pathogenesis and management of pediatric multifocal PVNS is warranted.