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The great mimickers: Castleman disease.

David Bonekamp1, Ralph H Hruban2, Elliot K Fishman1

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Summary

Castleman disease (CD) is a rare disorder often mimicking other conditions. Understanding its diverse presentations and associations is crucial for accurate diagnosis and treatment.

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Area of Science:

  • Oncology
  • Pathology
  • Immunology

Background:

  • Castleman disease (CD) presents as localized or generalized lymphadenopathy.
  • It frequently mimics benign and malignant masses, posing diagnostic challenges.
  • CD is associated with various neoplasms, skin diseases, and immunodeficiencies.

Purpose of the Study:

  • To review the diverse clinical presentations of Castleman disease.
  • To highlight diagnostic challenges and differential diagnoses.
  • To discuss the pathogenesis and treatment strategies for CD.

Main Methods:

  • Literature review of Castleman disease presentations.
  • Analysis of diagnostic imaging features.
  • Summary of associated conditions and treatment modalities.

Main Results:

  • Classic hyaline-vascular CD typically shows unicentric lymphadenopathy.
  • Multicentric forms, including HHV8-associated CD, present with generalized adenopathy and systemic symptoms.
  • CD is linked to cytokine overproduction, particularly IL-6.

Conclusions:

  • Castleman disease requires a high index of suspicion due to its mimetic nature.
  • Accurate diagnosis involves recognizing diverse clinical and imaging findings.
  • Treatment varies from surgical resection to systemic therapies targeting IL-6.