[Castleman disease]. / Enfermedad de Castleman.

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection.

Enfermedad de Castleman / Castleman disease

La enfermedad de Castleman o hiperplasia angiofolicular es una entidad poco frecuente enmarcada dentro de los trastornos linfoproliferativos. Esta enfermedad fue descrita inicialmente por Castleman en 1956. Su etiología es desconocida, pero se cree que responde a una estimulación antigénica crónica de probable etiología viral. Se presenta en adultos jóvenes, más frecuente en mujeres, y es excepcional en la edad pediátrica. Clínicamente se distinguen dos formas de enfermedad: una localizada y otra multicéntrica. La forma localizada, de buen pronóstico, asintomática al diagnóstico, es la forma de presentación más frecuente en la edad pediátrica y por lo general corresponde a masas mediastínicas muy vascularizadas. La resección de la lesión, que es curativa, se asocia a un alto riesgo de sangrado. Recientemente se ha introducido la arteriografía con embolización prequirúrgica para minimizar el riesgo de sangrado en el acto quirúrgico. Se presenta un caso de enfermedad de Castleman localizada en una paciente de 12 años en la que se utilizó con éxito la angioembolización prequirúrgica

Castleman disease or angiofollicular hyperplasia is a rare disorder included in the group of lymphoproliferative disorders. This entity was originally described by Castleman in 1956. The etiology remains unknown but it is postulated to be a reactive lymphoid hyperplasia due to chronic antigenic stimulation caused by a viral infection. The disease presents in young adults and is more frequent in women; it is exceptionally rare in the pediatric age group. It is classified into two clinical groups (localized disease and disseminated disease) and there are two histologic variants (hyaline-vascular and plasma cell Castleman disease). Localized disease is usually asymptomatic, has a good prognosis, and is the most common presentation in pediatric patients, usually corresponding to highly vascularized mediastinal masses. Resection of the mass, which is curative, is associated with a high risk of blood loss. Recently, preoperative arteriography with embolization has been used satisfactorily in the preoperative management of these tumors. We present a case of localized Castleman disease in a 12-year-old girl satisfactorily treated with embolization before curative resection