Rhabdomyosarcoma (RMS) is a malignant tumor of immature mesenchymal cells; it can arise from any part of the body. RMS is the most common soft tissue sarcomain the childhood and its most frequent primary site is the head and neck. AIM: The purpose of this study was to review the occurrence of head and neck RMS in childhood and its clinical-histopathologic features. STUDY DESIGN: Clinical retrospective. METHOD: Eighty-two patients with soft tissue sarcoma treated in the Institute of Pediatric Oncology, UNIFESP-EPM, between 1988 and 2002 were included in this study. The medical records of the patients with head and neck soft tissue sarcomas were reviewed. The following parameters were analyzed: incidence of RMS in the childhood and in the head and neck, sex, age, histological classification, primary site, follow-up. RESULTS: In this study, 65% of soft tissue sarcomas were RMS; 33% of RMS were in the head and neck region; 77% of soft tissue sarcoma in this region were RMS. The mean age at diagnosis was 7.62 years, 41% were between 5 and 9 years. In this sample, there were 47% men and 53% women. Embryonic RMS was the predominant histological type (64.6%) and the orbit was the most common primary site (52.8%). One hundred percent, 50% and 33.3% of the parameningeal, nonparameningeal and orbital RMS, respectively, led to death. CONCLUSION: RMS is the most common soft tissue sarcoma in the childhood and the head and neck is the most frequent primary site. Males were more affected by RMS than females. The mean age at diagnosis was 7.62 years and the most frequent age interval was between 5 and 9 years old. The embryonic histological type and the orbit as primary site were predominant. The highest mortality was found for parameningeal RMS and the lowest, for orbital RMS.
rhabdomyosarcoma; pediatric oncology; head and neck neoplasm
