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Castleman's disease in children.

R W Powell, A L Lightsey, W J Thomas

    Journal of Pediatric Surgery
    |August 1, 1986
    PubMed
    Summary
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    Castleman's disease, a rare lymph node disorder, presents differently in children. Surgical resection offers a cure for both symptomatic and asymptomatic forms, with no reported recurrences in pediatric patients.

    Area of Science:

    • Pediatric Oncology
    • Hematology
    • Rare Diseases

    Background:

    • Castleman's disease (CD), also known as giant lymph node hyperplasia, is a rare lymphoproliferative disorder.
    • It typically affects adults but can occur in children, presenting in distinct clinical forms.
    • Understanding pediatric CD is crucial due to its rarity and potential to mimic malignancy.

    Observation:

    • Two pediatric cases of Castleman's disease are presented: a 3.5-year-old girl with symptomatic plasma-cell type and a 12-year-old boy with asymptomatic hyaline-vascular type.
    • The girl exhibited anemia, fever, night sweats, hypergammaglobulinemia, and an abdominal mass.
    • The boy presented with acute appendicitis, with a hilar mass incidentally found on chest radiograph.

    Findings:

    • Both pediatric patients achieved complete remission following surgical resection of affected lymph nodes.

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  • The symptomatic case represents the youngest reported patient in the English literature.
  • No recurrences were observed in either patient four years post-surgery.

    • Implications:

    • Surgical resection is the primary diagnostic and therapeutic approach for pediatric Castleman's disease.
    • Early diagnosis and management are essential, as enlarged lymph nodes can be mistaken for lymphoid malignancies.
    • This study highlights the benign nature and favorable prognosis of Castleman's disease in children after complete resection.