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  • Title: [Soft tissue sarcomas (1991-2004)].
    Author: Andrés AM, Avila LF, Luis AL, Encinas JL, Sastre A, López-Gutiérrez JC, Martínez L, Queizán A, Martínez-Urrutia MJ, Jaureguizar E, Tovar JA.
    Journal: Cir Pediatr; 2006 Oct; 19(4):210-6. PubMed ID: 17352109.
    Abstract:
    BACKGROUND: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years. MATERIAL AND METHODS: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests. RESULTS: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y). Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 +/- 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary location, non radical excission, the presence of distant metastases at onset and alveolar histology. 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years). Among this group, there were 6 fibrosarcomas, 4 indifferentiated sarcomas, 3 synovial sarcomas, 2 abdominal desmoplastic small round cell tumours, 2 neurofibrosarcomas and 1 leiomyosarcoma. Only 9 received chemotherapy and one radiotherapy. All but one were operated. Five out ot the 19 died. CONCLUSIONS: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct. The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.
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