Castleman Disease with Endocrine Dysfunction

Abstract

Castleman disease is a rare, non-clonal lymphoproliferative disorder predominantly affecting lymphoid tissues. This report presents the case of a 23-year-old male with a chronic left-sided neck swelling and gynecomastia. Surgical excision and Webster technique for gynecomastia were performed, and histopathological examination revealed features consistent with Castleman disease. The lymph node specimen displayed enlarged and reactive atretic germinal centers with an onion skin arrangement of lymphocytes and occasional atypical follicular dendritic cells. Additionally, the breast tissue showed proliferating ducts indicative of gynecomastia. Immunohistochemical analysis confirmed the diagnosis, revealing CD20-positive mantle zones, diffuse CD45 positivity, CD3 positivity in the paracortex and interfollicular area, and Ki-67 positivity in the germinal center. Castleman disease presents clinically as unicentric or multicentric forms and histologically as hyaline vascular, plasma cell, mixed cellular, or plasmablastic variants. Elevated levels of vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6) underscore the disease's inflammatory background. The disease's heterogeneity presents challenges in diagnosis, particularly the plasma cell variant, which can mimic Hodgkin lymphoma. Laboratory abnormalities, such as elevated ESR, LDH, CRP, and anemia, are seen in the multicentric variant. Associations with POEMS syndrome, Kaposi's sarcoma, and co-existing malignant lymphomas complicate the disease's clinical course. Recognition of this condition is crucial for timely intervention, as surgical excision is curative in unicentric cases, while multicentric forms often exhibit a rebound effect. Understanding the disease's diverse presentations and associated lymphomas is essential for accurate diagnosis and appropriate management.