Resumo

GIMENEZ, CESAR  e  HOSPITAL DE NINOS “J M DE LOS RIOS”, CARACAS, VENEZUELA et al. Sarcomas de tejidos blandos  no rabdomiosarcoma en niños. Rev. venez. oncol. [online]. 2007, vol.19, n.3, pp.230-234. ISSN 0798-0582.

OBJECTIVE: Determine the incidence, epidemiology and the prognostic factors in children with non-rhabdomyosarcoma soft-tissue sarcoma. METHODS: 38 patients were reviewed obtaining itself data related: Age, sex, anatomic site, tumor size, immunohistochemistry, metastases, treatment and present status. RESULTS: 1995-2005 were 38 cases. 21 males average 8.5 years (rank 4m-16 years). 65.78 % required immunohistochemical for diagnosis definitive. Biopsy by aspiration with fine needle was made in 39.47 % without obtaining histological diagnosis. Malignant peripheral nerve sheath tumor represented 31.57 % fibrosarcomas (18.42 %). The most common anatomic site affected were extremities (39.47 %) followed by chest wall (26.31 %). 26 patients (68.42 %) reached complete remission with surgery and 4 with surgery plus chemotherapy and/or x-ray. There was no metastatic disease. 65.78 % were malignant. 18 patients (47.27 %) had tumors ≥ 5cm. There were 2 local relapses in patients with incomplete surgeries. 28 stages I, 9 II 1 ST III. 5 died by progression 3 are alive with disease. 78.24 % are alive without disease at a median follow-up of 5.16 years. CONCLUSIONS: The incidence of non-rhabdomyosarcoma soft-tissue sarcoma in our center was 4.2 %. There is a slight male predominance. The extremities were the primary site more affected. Immuno-histochemestry is fundamental for diagnosis. BAAF was not useful and delayed the diagnosis. Intraabdominal tumors, high grade tumors and tumors ≥ 5 cm were adverse factors. The main treatment is the surgery and chemotherapy, radiotherapy allowed rescuing some patients.

Palavras-chave : Cancer; soft tissue sarcomas; rabdomyosarcomas.