Abstract:

Objective To analyze clinical features of Castleman disease (CD) for improving clinicians' understanding of CD. Methods Data including clinical manifestation, diagnosis, therapy and prognosis of pediatric patients with CD diagnosed and treated in a single center in Children's Hospital of Chongqing Medical University from January, 2010 to June, 2020 were retrospectively analyzed. Results There are 21 children with CD (11 males and 10 females), the median age of patients was 10.90 (5.35, 12.1) years old. All patients have performed CT examinations. Among them, all unicentric CD (UCD) patients underwent complete surgical resection, and six patients of multicentric CD (MCD) were treated with chemotherapy, anti-IL-6 and hormones. The median follow-up time of 21cases is 37 months (27,71), 2 UCD patients died with paraneoplastic pemphigus and lung interstitial changes. Conclusion Castleman disease is rare in pediatric patients, and the overall prognosis is well if it was treated timely. For UCD patients, preferred surgical treatment for asymptomatic UCD children have high cure rate. Children with MCD treated with anti-IL-6, hormones, and chemotherapy have significantly improved long-term survival rates.

Key words: Castleman disease, clinical manifestation, therapy, prognosis