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

Castleman's disease (CD) is a rare and not well recognized disease characterized by a massive growth of lymphoid tissue of unknown etiology that was first described in 1954, and subsequently better defined by Castleman in 1956 [1,2]. The disease usually presents in young adults and is probably slightly more frequent in women. CD is classified into two clinical subtypes: a localized and a multifocal subtype. CD may occur anywhere along the lymphatic system, although the most common location (70%) is the mediastinum. Extrathoracic sites have been reported in the neck, axilla, pelvis and retroperitoneum [3]. Surgery is the optimal therapeutic approach only in the localized form, while for unresectable or disseminated disease, partial surgical resection, steroid, chemotherapy and radiotherapy have been employed with some measurable success.We report three cases of localized hyaline-vascular CD in pediatric age observed in our Department in the last 3 years; a complete surgical resection was not feasible in one of them and so various therapeutic attempts were performed.GM, female, was admitted, at the age of 3.3 years, with an asymptomatic left axillary mass showing a slow progressive growth. At presentation she was in good general conditions and the physical examination was normal. Routine blood tests were all normal too. Radiological examinations (chest X-ray, abdominal ultrasound, chest CT scan) confirmed the adenopathy and excluded other locations. After 2 months a complete surgical resection was performed, and a single lymph node of 4 cm maximum diameter was removed. Histological assessments indicated the diagnosis of hyaline-vascular CD (Figure 1A,B). There has been no recurrence 48 months after diagnosis.LG, male; at the age of 3.8 years an isolated palpable subcutaneous tumefaction, sized 1 cm in maximum diameter, appeared on his right blade shoulder. On admission he was in good physical condition; routine blood tests, abdominal ultrasound and chest X-ray were al