RESUMO
Os autores relatam um caso de condrossarcoma em uma paciente de 14 anos portadora de osteocondromatose múltipla, que procurou assistência médica com a queixa de tumoração na coxa esquerda. Cortes tomográficos do membro afetado evidenciaram lesão osteodestrutiva no terço superior do fêmur esquerdo. O diagnóstico foi feito através da revisão da lâmina do serviço de origem da paciente, confirmando o achado de condrossarcoma grau I em lesão condromatosa. Foi realizada desarticulação do colo femoral devido ao extenso comprometimento de grupos musculares, de tecido celular subcutâneo e de pele. A literatura mostra ampla variação nas taxas de transformação maligna das exostoses em pacientes portadores desta doença, sendo esta uma importante complicação nestes pacientes.
The authors report a case of chondrosarcoma in a 14-year-old female patient with multiple osteochondromatosis who has sought medical assistance complaining of a tumor on the left coxa. CT images of the affected limb have demonstrated osteodestructive lesion on the superior third of the left femur. The diagnosis has been made through a review of the biopsy slide from the service of origin of the patient, confirming the finding of a grade I chondrosarcoma in a chondromatous lesion. Disarticulation of the femoral neck was made because of the large extent of the lesion in muscular groups, subcutaneous cellular tissue and skin. The literature shows a wide variation in rates of malignant transformation of exostosis in patients with this disease, and this is a significant complication in patients affected by this disease.
Assuntos
Humanos , Feminino , Adolescente , Neoplasias Ósseas , Condrossarcoma , Condrossarcoma/complicações , Condrossarcoma/diagnóstico , Fêmur , Osteocondromatose/diagnóstico , Osteocondromatose/etiologia , Fêmur/anatomia & histologia , Fêmur , Tomografia Computadorizada por Raios XRESUMO
A case of tenosynovial osteochondromatosis in the tarsal tunnel in a 23-year-old man is presented. The lesion was treated surgically, and multiple osteochondromas were excised, which had no continuity with any tarsal bone or joint cavity but did with the sheaths of the flexor digitorum longus and flexor hallucis longus. Histologic examination of the lesion showed a fibrous capsule, hyaline cartilage and extensive areas of cancellous bone. Necrosis and mitosis were absent in the hyaline cartilage and there were no synovial nodules indicative of synovial metaplasia. The macroscopic findings showed "end-stage" tenosynovial osteochondromatosis. There was no evidence of recurrence 5 years after operation, and the patient remains free of symptoms.
Assuntos
Tornozelo , Condromatose Sinovial/diagnóstico , Osteocondromatose/diagnóstico , Ossos do Tarso/lesões , Adulto , Condromatose Sinovial/etiologia , Condromatose Sinovial/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Osteocondromatose/etiologia , Osteocondromatose/cirurgia , Esqui/lesões , Ossos do Tarso/patologia , Ossos do Tarso/cirurgia , Tomografia Computadorizada por Raios XRESUMO
Nine patients developed osteochondromata, a mean of 6 years after total body irradiation (TBI) given before bone marrow transplantation for childhood leukaemia. This represents 23% of patients receiving TBI during the period from 1981 to 1989 surviving > or =5 years after bone marrow transplantation. The patients were followed up for a mean of 12.5 years from diagnosis of leukaemia and a mean of 2.5 years from diagnosis of osteochondromata. No osteochondroma, including three lesions removed surgically, showed evidence of malignant change. Six patients received growth hormone for irradiation-induced growth hormone deficiency, but this did not appear to influence the natural history of the osteochondromata. Radiation-induced osteochondromata (RIO) are often multiple and are indistinguishable from the more common idiopathic type. The incidence of RIO after TBI was higher than that reported after local irradiation.