Abstract
Objective: Review the clinical presentation, histological subtypes, staging, treatment modalities, and survival rates for non-orbital pediatric head and neck rhabdomyosarcoma (RMS).
Method: A retrospective chart review of patients treated from 1996 to 2011 for nonorbital primary head and neck RMS at a pediatric tertiary care center. Baseline demographics, clinical presentation, staging, histology, treatment modalities, and survival outcome were analyzed.
Results: Sixteen patients (7 M) were diagnosed with primary head and neck RMS, median age of 6.9 years (range, <1-19 years). The majority of tumors were of parameningeal location (11/16). Fifty-six percent (9/16) were metastatic at presentation (stage at diagnosis: 1, n = 1; 2, n = 2; 3, n = 4; 4, n = 9). Nine had skull base erosion and/or cranial nerve deficits. Ten patients had embryonal and sihad alveolar histology. Upfront resection gross resection was feasible in 4 patients (3 of which were non-parameningeal), all with positive margins. All patients received chemotherapy combined with radiotherapy. Second look surgery was performed in 2 patients.
Conclusion: Most patients presented with advanced disease in our series. Majority were parameningeal, thus limiting the role to biopsy only. Primary excision for non-paramenigeal RMS is ideal. The role of second look surgery after chemotherapy and radiation is still unknown; however, it was successful in 2 patients in our small review.
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