Castleman's disease in childhood: report of three cases and review of the literature

Abstract

Castleman's disease (CD) is a rare, localized or generalized, lymphoproliferative disorder with a frequent mediastinal location, but possible in any lymph node or extra nodal site. It usually appears in young adults whilst it rarely occurs in childhood. There are only about 100 pediatric cases published, five of them in Italy. We report 3 cases of localized Castleman's disease, investigated in our Department in a 3 years period and reviewed the literature.

Figure 1

Histology showed a follicular center exihibiting prominent hyalinized vessels, surrounded by concentric layers of lymphocytes (A), dendritic follicular cells in the germinal center were positive for CD21 (B).

Figure 2

Histology showed small hyalinized germinal center within an expanded mantle zone (A), dendritic follicular cells in the germinal center were CD21-positive (B).

Figure 3

CT scan showed that the neck mass, a multiple lymph node enlargement, with an high contrast-enhancement, originated in the anterior-superior mediastinum and cause a serious right dislocation of the trachea (A-D).

Figure 4

Histology showed small germinal center depleted of follicular center B cells, and penetration by hyalinized venules (A); follicular dendritic cell were evidenced by their strong immunoreactivity for CD 35 (B); immunoreactivity for CD20 (C) and CD3 (D) was strongly positive.