Haga su diagnóstico / Make your diagnosis

El diagnóstico es Osteocondromatosis Multiple Hereditaria

Cambios metafisiarios de los miembros inferiores originados por osteocondromatosis múltiple hereditaria / Mataphyseal changes of the lower limb caused by multiple hereditary osteochondromatosis

Se realizó un estudio de población, de tipo prospectivo, descriptivo y observacional en pacientes que acudieron a la Unidad de Tumores Óseos y Partes Blandas (UTOPB) del Hospital Universitario “Dr. Manuel Núñez Tovar” entre enero de 2008 y julio de 2010, con el diagnóstico de Osteocondromatosis Múltiple Hereditaria (OMH) que afectaba las articulaciones de las extremidades inferiores; se estudiaron 26 pacientes, equivalentes a 52 extremidades inferiores, con predominio del sexo masculino 1,2:1, las edades de los pacientes comprendían entre los 6 y 15 años, con mayor registro de casos a los 12 años (19,2%). Se cuantificó el número de osteocondromas periarticulares en las articulaciones de cadera, rodilla y tobillo, observando un predominio en la rodilla (48,7%) a expensas de la metáfisis distal del fémur. Se clasificó la afectación funcional de las articulaciones en tres grados (leve, moderada y severa), tomando como referencia el movimiento de flexoextensión, siendo el tobillo la articulación que presento el mayor grado de limitación y la cadera la menos afectada a la presencia de osteocondromas

We conducted a prospective, descriptive and observational population-based study, in patients attending the Unit of Bone and Soft Tissue Tumors (UTOPB) at the Hospital "Dr. Manuel Núñez Tovar" between January 2008 and July 2010, with the diagnosis of Multiple Hereditary Osteochondromatosis (MHO), which affected the joints of the lower extremities, were studied 26 patients, equivalent to 52 lower extremities; with a male predominance 1,2:1, the age of the patients ranged between 6 and 15 years, with highest number of cases at 12 years (19.2%). We quantified the number of periarticular osteochondromas at the hip, knee and ankle, having predominance in the knee (48.7%) at the expense of the distal femur metaphysis. We scored the functional range of the joints in three grades (mild, moderate and severe), with reference to the movement of flexion-extension, the ankle joint had the highest degree of limitation and, the hip was less affected to the presence of osteochondromas

Condrossarcoma em paciente com osteocondromatose múltipla: relato de caso e revisão da literatura / Chondrosarcoma in a patient with multiple osteochondromatosis: a case report and review of the literature

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.