ABSTRACT
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.
Subject(s)
Castleman Disease , Castleman Disease/pathology , Castleman Disease/surgery , Child, Preschool , Female , HumansABSTRACT
La enfermedad de Castleman es un trastorno linfoproliferativo de etiología desconocida, de escasa frecuencia en Pediatría (sólo hay 100 casos descritos, 7 en España). Suele afectar a adultos jóvenes y se describen 2 formas clínicas: la forma localizada (que suele presentarse como una masa única y asintomática al diagnóstico) y la forma multicéntrica, (generalmente con afectación sistémica y un comportamiento más agresivo). El diagnóstico de confirmación es anatómico y patológico. Histológicamente hay 2 variantes: la forma hialina-vascular, que suele corresponder a las formas localizadas de la enfermedad, y la variante de células plasmáticas, que suele ser multicéntrica. La resección quirúrgica es curativa para las formas localizadas, mientras que se han ensayado una multitud de tratamientos sin éxito para las formas multicéntricas. A continuación se presenta el caso de una preescolar de 3 años con una forma localizada de la enfermedad que, tras 2 años de seguimiento, se mantiene asintomática. Asimismo, se realiza una revisión de la literatura médica disponible al respecto (AU)
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 (AU)
Subject(s)
Humans , Female , Child, Preschool , Castleman Disease/pathology , Castleman Disease/surgeryABSTRACT
OBJECTIVE: The purpose of this study was to evaluate the use of sonographically guided directional vacuum-assisted biopsy in the histologic diagnosis of breast lesions. MATERIALS AND METHODS: Eighty-eight lesions in 83 women underwent sonographically guided 11-gauge directional vacuum-assisted breast biopsy during a 26-month period. Biopsies were performed using high-resolution sonography equipment with a 7.5-MHz transducer, obtaining a median of 17 specimens per lesion. Imaging studies, medical records, and histologic findings were reviewed. RESULTS: Median patient age was 48 years (range, 25-78 years). Median lesion size was 1.2 cm (range, 0.4-2.5 cm). Twenty-four (27.3%) of 88 lesions were palpable. The median time required to perform biopsy was 17 min (range, 10-40 min). Complete removal of the lesion seen at sonography occurred in 78 (88.6%) of 88 lesions and was significantly more frequent in lesions measuring 1.5 cm or less than in larger lesions (68/71 = 95.8% vs 10/17 = 58.8%,p < 0.0003). A surgical procedure was spared in 79 (95.2%) of 83 women. In 36 lesions with imaging and clinical follow-up after sonographically guided biopsy with benign findings (range, 4-24 months; median, 11.3 months), we found no evidence of cancer or scarring in the breast. CONCLUSION: In our small series, sonographically guided directional vacuum-assisted biopsy was a fast and accurate method for breast diagnosis. This technique resulted in complete removal of 95.8% of lesions shown at sonography measuring 1.5 cm or less and spared a surgical procedure in 95.2% of women. Further work is necessary to refine indications, evaluate cost-effectiveness, and assess long-term outcome.