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Vol. 10. Issue 1.
Pages 29-34 (March 2009)
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Vol. 10. Issue 1.
Pages 29-34 (March 2009)
Revisión
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Sarcomas sinoviales
Synovial sarcomas
Visits
10255
Manuel Bravo-Péreza, Susana López-Lópezb, Carlos García-Porrúac,
Corresponding author
cgporrua@hotmail.com

Autor para correspondencia.
a Servicio de Cirugía Ortopédica y Traumatología, Hospital Xeral-Calde, Lugo, España
b Servicio de Urgencias, Hospital Xeral-Calde, Lugo, España
c Sección de Reumatología, Hospital Xeral-Calde, Lugo, España
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Resumen

Los sarcomas sinoviales representan entre un 5 y un 10% de los sarcomas de partes blandas. No obstante, son muy agresivos y el 10% de los pacientes fallece en el primer año tras el diagnóstico. Suelen afectar a adolescentes y adultos jóvenes con edades comprendidas entre los 15 y los 40 años. La presentación clínica habitual es la de una masa yuxtaarticular palpable, generalmente dolorosa. No obstante, debido al bajo índice de sospecha, se puede confundir con tumoraciones benignas de partes blandas, bursitis o artritis, lo que favorece el retraso del diagnóstico, y no son raras demoras en el diagnóstico de más de un año. Puede localizarse en cualquier parte del organismo. Sin embargo, la localización más habitual en las extremidades. La supervivencia varía según el tamaño y la localización tumoral, la afectación profunda, el grado histológico y la presencia de afectación ganglionar o metástasis. En prácticamente todos los casos contienen una translocación característica entre los cromosomas X y 18. Esta translocación fusiona el gen SYT del cromosoma 18 a uno de sus genes homólogos (SSX1, SSX2 y SSX4) localizados en el cromosoma X. La cirugía es el tratamiento de base, acompañada, en ocasiones, de radioterapia y quimioterapia.

Palabras clave:
Sarcoma sinovial
Manifestaciones clínicas
Pronóstico
Abstract

Synovial sarcomas represent 5-10% of all soft tissue sarcomas. Nevertheless, these tumors are highly aggressive, causing death in 10% of affected individuals in the first year after diagnosis. Synovial sarcomas are more prevalent in adolescents and young adults aged between 15 and 40 years old. The most common clinical presentation is a generally painful, juxta-articular, palpable mass. Due to the low index of suspicion, synovial sarcoma is often misdiagnosed as benign soft tissue processes, bursitis or arthritis and diagnosis can be delayed for more than 1 year. These tumors can occur in any site but most arise from deep soft tissues in the lower extremities. Survival rates vary significantly according to tumoral size and location, depth, histologic grade, and the presence of nodal involvement or distant metastasis. In almost all cases, synovial sarcomas contain a characteristic translocation between chromosomes X and 18. This translocation involves the SYT gene on chromosome 18 and one of several highly homologous genes (SSX1, SSX2 and SSX4) on the X chromosome. The cornerstone of treatment is surgery, along with radiation therapy and chemotherapy.

Keywords:
Synovial sarcoma
Clinical features
Prognosis
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