Abstract
BackgroundTo describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature.Material and MethodsCases diagnosed as RMS affecting the head and neck region were retrospectively retrieved from the files of two Brazilian institutions from January 2006 to January 2017. Data on clinical features (sex, age and affected site), microscopic subtype, immunohistochemical results, treatment employed and follow-up status were obtained from the patient’s medical charts.ResultsDuring the period considered, 10 cases of RMS were identified. Females predominated (4M:6F), the mean age at diagnosis was 16.5 years-old and the orbit was the most affected site (4 cases). Microscopically, most cases were classified as embryonal RMS (6 cases) and the Desmin/Myogenin/Myo-D1 immunohistochemical positivity was useful to confirm the diagnosis. Chemotherapy and radiotherapy were applied to 9 and 8 patients respectively, whereas 2 patients were treated by surgery. Recurrences occurred in 3 patients and distant metastasis in 2 cases. Nine patients were alive in their last follow-up, 3 of them with disease, whereas 1 patient died due to the disease.ConclusionsHead and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment. Key words:Rhabdomyosarcoma, soft tissue tumors, head and neck, oral cavity, chemotherapy.
Highlights
Rhabdomyosarcoma (RMS) is classified by the World Health Organization as a skeletal muscle tumor arising from undifferentiated skeletal tissue (1,2), predominantly affecting the head and neck region, with approximately 40% of the cases involving this area (3-5)
In this study we described a series of 10 cases of head and neck RMS and reviewed all published clinical series dealing with RMS of the head and neck with at least 3 cases reported to better understand the clinicopathological features of this aggressive malignancy (Table 2,2 continue, 2 continue-1, 2 continue-2, 2 continue-3.)
RMS can be classified into different histologic subtypes, and as shown in our study, the most common is the embryonal subtype (EMB), accounting for 60% of all cases, characterized by undifferentiated, small, round and hypercromatic cells with variable number of strap or tadpole-shaped, eosinophilic rhabdomyoblasts (11,20)
Summary
Rhabdomyosarcoma (RMS) is classified by the World Health Organization as a skeletal muscle tumor arising from undifferentiated skeletal tissue (1,2), predominantly affecting the head and neck region, with approximately 40% of the cases involving this area (3-5). RMS is the most common soft tissue sarcoma in children, accounting for 4.5% of all pediatric malignant neoplasms and approximately 50% of the solid malignancies diagnosed in patients under 10 years old (6). RMS are highly sensitive to chemotherapy and radiotherapy, as a consequence, over the last 30 years pediatric patients had a significant improvement in their prognosis, with the 5-year survival rates achieving 80% to 85% in some series (4,8,9). The outcome for adults is not as satisfactory as for the pediatric patients and both children and adults are currently treated by aggressive surgical resections followed by chemotherapy and radiotherapy (10). Conclusions: Head and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment
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