RESUMO
Fibrocartilaginous dysplasia is a variant of fibrous dysplasia in which extensive cartilaginous differentiation is identified. The amount of cartilage varies from case to case, however, no percentage has been proposed to consider this diagnosis. We present a 6-year-old girl with a two-year history of hip pain. Initial imaging studies of the right femur revealed a lucent lesion of the proximal shaft that extended into the femoral neck with ill-defined borders but well maintained cortex. Computed tomography scan demonstrated increased density of the medullary cavity but the cortex appeared intact. Curettage of the lesion was performed and fragments with cartilaginous appearance were obtained, weighing 45 g in total. Microscopically, the tumor revealed a cartilaginous (60%) and a fibro-osseous (40%) component; the former had increased cellularity and some chondrocytes displayed moderate atypia and binucleation, while the latter showed features of fibrous dysplasia. Areas of endochondral ossification and calcification were also identified. After five years of surgery this child is well and without evidence of recurrence. We discuss the differential diagnosis of this variant of fibrous dysplasia in the pediatric group.
Assuntos
Cartilagem/patologia , Displasia Fibrosa Óssea/diagnóstico , Displasia Fibrosa Óssea/patologia , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/patologia , Cartilagem/diagnóstico por imagem , Diferenciação Celular , Criança , Condrócitos/patologia , Condrossarcoma/diagnóstico , Condrossarcoma/patologia , Diagnóstico Diferencial , Feminino , Displasia Fibrosa Óssea/diagnóstico por imagem , Humanos , Tomografia Computadorizada por Raios XRESUMO
Giant cell fibroblastoma (GCF) is a rare soft tissue tumor most often discovered during the first two decades of life. We present a case of a 3-year-old boy with a history of a recurrent lesion in the knee, the tumor growth progressively and enlarged to 2.1 cm in the previous two years before diagnosis. It involved the subcutaneous tissue, had infiltrative borders and extended into the superficial dermis. The tumor was surgically excised with free margins. There was no evidence of local recurrence, and a metastatic workup was negative after 10 years of follow up. We review herein the clinicopathologic features, histogenesis, differential diagnosis and relationship to dermatofibrosarcoma protuberans (DFSP).
Assuntos
Dermatofibrossarcoma/patologia , Tumores de Células Gigantes/patologia , Neoplasias Cutâneas/patologia , Neoplasias de Tecidos Moles/patologia , Pré-Escolar , Dermatofibrossarcoma/ultraestrutura , Diagnóstico Diferencial , Tumores de Células Gigantes/ultraestrutura , Humanos , Imuno-Histoquímica , Joelho/patologia , Masculino , Neoplasias Cutâneas/ultraestrutura , Neoplasias de Tecidos Moles/ultraestruturaRESUMO
Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. Mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. Autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglobinopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.