RESUMO
INTRODUCTION: Rhabdomyosarcoma (RMS) is a malignant solid tumor of mesenchymal origin. It is the most com mon soft-tissue sarcoma in childhood and adolescence. 65% of cases are diagnosed before the age of 6. Histological subtypes include embryonal, alveolar, pleomorphic, and fused-cell RMS. The embryo nal subtype is more frequent in children, while the alveolar one is more frequent in adolescents and adults. OBJECTIVE: To describe the clinical presentation of primary alveolar rhabdomyosarcoma in a schoolgirl. CLINICAL CASE: 7-year-old schoolgirl with one-month history of progressive pain in her left thigh. X-ray shows a lytic lesion in the left femur diaphysis. A study was performed with 2 biopsies, immunohistochemistry, and PAX-FOXO1 studies which were compatible with alveolar RMS. Con clusion: Primary alveolar rhabdomyosarcoma of the bone is rare, but it should be considered within the differential diagnosis of primary small-round-blue cell bone tumors. Despite presenting a poor prognosis cytogenetic, this type of tumor seems to have better biological behavior, which for a successful treatment makes necessary to have a high index of suspicion in order to install a multimodal therapy in the context of a national protocol.
Assuntos
Neoplasias Femorais/diagnóstico , Rabdomiossarcoma Alveolar/diagnóstico , Criança , Feminino , Neoplasias Femorais/patologia , Humanos , Rabdomiossarcoma Alveolar/patologiaRESUMO
Pelvic resection surgeries due to primary bone tumors are infrequent. According to the compromised area we can divide it in three zones, being those who affect the acetabulum the ones with the major functional alterations, pelvic stability and leg length discrepancy. The aim of this study is to present our experience in the surgical management of this type of lesions. From series of 17 patients, eight (47 percent) presented complications, the most frequents was surgical wound infection (23 percent) and deep venous thrombosis (18 percent). 4 patients died (23 percent). The other 13 patients answered the Musculo Skeletal Tumor Society (MSTS) scale being the average score 22,3. One local recurrence appeared in this group. The acceptance of the patients in this kind of surgeries is very good. These surgeries are complex, there is a high rate of complications. They must be performed in centers with a suitable professional and technical equipment.
Las cirugías de resecciones pélvicas por tumores óseos primarios son infrecuentes. Según el área comprometida se divide principalmente en 3 zonas, siendo las que afectan al acetábulo las que producen mayor alteración (funcional, en la estabilidad pélvica y la longitud de las extremidades inferiores). El objetivo de este trabajo es presentar la experiencia del equipo de tumores del Instituto Traumatológico y la Clínica Santa María en el manejo quirúrgico de este tipo de lesiones. Con un total de 17 pacientes, 8 presentaron complicaciones (47 por ciento), la más frecuente fue la infección de herida operatoria (4 pacientes 23 por ciento) y la trombosis venosa profunda (3 pacientes 18 por ciento). Cuatro pacientes (24 por ciento) fallecieron, los 13 restantes respondieron la escala Musculo Skeletal Tumor Society (MSTS) siendo el puntaje promedio de 22,3. Se presentó una recidiva local en este grupo. El grado de aceptación de los pacientes es muy bueno. Deben realizarse en centros con el equipamiento profesional y técnico adecuado para las exigentes y complejas cirugías a realizar.