Clinical and experimental study of Castleman disease in children.

BACKGROUND Castleman disease (CD) is a rare lymphoproliferative disease that is often underdiagnosed or misdiagnosed, especially in children. For this reason, we describe the clinical manifestations, diagnosis and treatment of CD in 11 children. PROCEDURE A retrospective study was performed to analyze the clinical features of 11 children with CD in a single institution from January 2001 to December 2012. All had computed tomography (CT) and lymph node resection for pathology diagnosis. RESULTS The average age of patients was 9.67 ± 4.26 years (range 1.3-15.5 years) including eight males (72.73%) and three females (27.27%). All but two (18.18%) had multicentric Castleman disease (MCD). Human immunodeficiency virus (HIV) or human herpes virus 8 (HHV8) infected cells were not detected in all patients. All patients were misdiagnosed in outside hospitals without tissue examination. Only in one case, the preoperative CT scan suggested CD. After treatment, 10 out of 11 children with CD in our study were disease free in the follow-up period ranging from 12 to 136 months (average 65.1 ± 10.21 months). CONCLUSION CD in children is rare, and is frequently misdiagnosed clinically. Our study shows that surgical resection is very effective in the treatment of unicentric Castleman disease (UCD). The rare UCD patient and all MCD patients treated with the modified NHLBFM-90 protocol had good prognosis.