ABSTRACT
OBJETIVO: Presentar un caso de radomiosarcoma paratesticular y revisión de la literatura. MÉTODOS: Describimos el caso de un varón de 16 años remitido a nuestro servicio por masa paratesticular izquierda de crecimiento progresivo, con imagen ecográfica de tumoración paratesticular heterogénea con flujo Doppler en su interior. RESULTADOS: Se realizó orquiectomía izquierda, con diagnóstico de rabdomiosarcoma. El paciente rechazó el tratamiento quimioterápico adyuvante. Tras 13 meses libre de enfermedad, reingresó por dolor cólico izquierdo detectándose en ecografía y TC una masa retroperitoneal paraaórtica izquierda que condicionaba uréterohidronefrosis grado III, y metástasis pulmonares. A pesar de instaurarse quimioterapia de rescate, no respondió presentando rápida progresión de la masa abdominal con importante deterioro general que no permitió el abordaje quirúrgico, siendo éxitus a las pocas semanas. CONCLUSIÓN: Los sarcomas paratesticulares son tumores infrecuentes y de mal pronóstico(AU)
OBJECTIVE: To report a case of paratesticular rhadomyosarcoma and to perform a bibliographic review. METHODS: We report the case of a 16-year-old male referred to our Department because of a left paratesticular hard tumor with progressive growth. Ultrasound examination showed a paratesticular heterogeneous mass with Internal flow on Doppler. RESULTS: The patient underwent left inguinal orchiectomy, with pathological diagnosis of rhabdomyosarcoma. He refused adjuvant chemotherapy. After being disease-free for 13 months, he presented with left colic pain. Ultrasound and CT examinations showed a left paraaortic retroperitoneal mass causing grade III ureterohydronephrosis, and lung metastases. Despite rescue chemotherapy treatment, there was no response and the abdominal mass progressed. A surgical approach was not possible since patient showed a rapid clinical worsening leading to his death a few weeks later. CONCLUSIONS: Paratesticular sarcomas are very uncommon tumors with poor prognosis(AU)
Subject(s)
Humans , Male , Young Adult , Myosarcoma/complications , Myosarcoma/diagnosis , Orchiectomy/instrumentation , Orchiectomy/methods , Testicular Neoplasms/complications , Testicular Neoplasms/diagnosis , Testicular Neoplasms/surgery , Pain/complications , Pain/diagnosis , Pain/etiology , Myosarcoma/physiopathology , Myosarcoma/surgery , Myosarcoma , Orchiectomy/standards , Orchiectomy/trends , Orchiectomy , Testicular Neoplasms/physiopathology , Testicular NeoplasmsABSTRACT
Myofibroblastic tumors are soft-tissue neoplasms arising from myofibroblasts, ubiquitous cells sharing ultrastructural features of muscular and fibroblastic cells. Vasudev and Harris described a malignant counterpart of these benign tumors in 1978. Most reported cases of myofibroblastic sarcoma have arisen in the head and neck region and the soft tissues of the extremities. To the best of the authors' knowledge, there have been only 8 previous reports on primary myofibroblastic sarcoma of the bone. The authors report a new case of this rare tumor affecting the sacrum and ilium of a 15-year-old girl and discuss the role of total sacrectomy and lumbopelvic reconstruction for treatment of this disease.
