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[Castleman's disease]

M Genoni1, D De Lorenzi, M Bogen

  • 1Ospedale San Giovanni, Bellinzona.

Deutsche Medizinische Wochenschrift (1946)
|September 17, 1993
PubMed
Summary
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A rare case of Castleman's disease presented as a periumbilical mass causing kidney obstruction in a teenager. Surgical removal led to symptom resolution, highlighting the importance of considering Castleman's disease in abdominal masses.

Area of Science:

  • Oncology
  • Pathology
  • Pediatric Surgery

Background:

  • Castleman's disease is a rare lymphoproliferative disorder with diverse clinical presentations.
  • Abdominal manifestations, particularly in pediatric cases, are infrequently documented.

Observation:

  • A 17-year-old female presented with acute abdominal pain, hematuria, and a palpable periumbilical mass.
  • Investigations revealed microcytic anemia, elevated ESR, positive Coombs test, left hydronephrosis, and a periumbilical tumor.
  • Imaging demonstrated a well-circumscribed, non-infiltrative tumor with specific contrast enhancement characteristics.

Findings:

  • Histopathological examination confirmed a mixed-type Castleman's disease with hyaline-sclerotic changes and inflammatory infiltrates.
  • The disease extended to involve the kidney, regional lymph nodes, and ureteral tissue, causing stenosis.

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  • Post-surgical treatment normalized inflammatory markers, although the Coombs test remained positive.

    • Implications:

    • This case underscores Castleman's disease as a potential cause of abdominal masses and obstructive uropathy in adolescents.
    • Multidisciplinary surgical and pathological approaches are crucial for accurate diagnosis and management.
    • Long-term monitoring is necessary due to potential recurrence or associated autoimmune phenomena.