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A clinical case of Castleman's disease
Joana Espiga Macedo1, Isabel Abreu, Margarida Marques
1Onco-hematology Service, Portuguese Institute of Oncology, Oporto, Portugal.
Abstract:
Angiofollicular lymph node hyperplasia, or Castleman's disease, is a rare disorder involving lymphoid tissue proliferation that was first described by Castleman in 1956. The etiology of Castleman's disease is unclear; many origins have been proposed, such as immunocompromised states, chronic inflammation or infection, and autoimmune processes. The disease has been classified on clinical grounds (unicentric or multicentric) and by histological appearance (hyaline vascular pattern, plasma cell predominance, or mixed lesions). In more than 70% of cases, Castleman's disease presents as a solitary mediastinal or cervical mass with an indolent course. Whereas the unicentric form is usually benign and curative resection is possible, patients with the multicentric form often have systemic symptoms and a clinically more aggressive, malignant course. We report a case of unicentric Castleman's disease and re-emphasize its importance in the differential diagnosis of mediastinal tumors.
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