Histologic and Laboratory Characteristics of Symptomatic and Asymptomatic Castleman Disease in the Pediatric Population

Objectives: Compare the morphologic, laboratory, and clinical features of asymptomatic and symptomatic Castleman disease in the pediatric population. Methods: We reviewed clinical records and histopathology of patients with Castleman disease from 2 pediatric institutions. Results: Of 39 patients with pediatric Castleman disease, 37 had unicentric disease, all classified with the hyaline vascular variant of Castleman disease, 8 of which were clinically symptomatic. These 8 patients demonstrated abnormal laboratory findings, including microcytic anemia, elevated erythrocyte sedimentation rate and C-reactive protein, and hypoalbuminemia. In addition, histopathologic evaluation showed that the 8 symptomatic cases had more hyperplastic germinal centers, fewer atrophic or regressed germinal centers, fewer mantle zones containing multiple germinal centers, reduced onion skinning of mantle zones, and fewer lollipop formations compared with the asymptomatic cases. Conclusions: This series of pediatric Castleman disease showed that lymph nodes from asymptomatic patients generally demonstrated the more classic hyaline vascular histology, whereas those with symptoms could lack or have only focal classic findings. As such, reactive lymph nodes with subtle Castleman-like features should prompt clinical correlation to ensure proper diagnosis.