[Castleman's disease: presentation of an atypical case]

Castleman's disease is an unknown etiology lymphoproliferative disorder rare in childhood (only 100 cases described in the literature, 7 of them in Spain). It usually appears in young adults, with no gender-related differences. There are two clinical forms of the disease: the localized variant, the more frequent type, usually presented as a solitary mass (generally in the mediastinum) and asymptomatic at the time of diagnosis, and the multicentric variant, less frequent and usually associated with systemic symptoms and a more aggressive clinical evolution. The diagnosis is confirmed based on the histopathological study. There are two histological variants: the hyaline-vascular type, which usually appears as a localised variant, and the plasma-cell type, which usually appears as multicentric variant and has a worse prognosis. While the best treatment for the localised variant is surgical resection, which is curative, for the multicentric variant many therapeutic strategies have been used without real success. In the present article we report a new case of this disease in a 3-year-old girl with the localised form, who remains asymptomatic after two years of follow-up, and a review of the available literature. This disease should be included in the differential diagnosis of the lymphoproliferative disorders.