Abstract
Tongue rhabdomyosarcomas (RMSs) are extremely rare soft tissue tumors in the pediatric age group. The most common reported histopathology type is embryonal. To our knowledge, epithelioid type has not yet been reported in tongue RMSs. We report a case of an eight-year-old boy who presented with a painless tongue mass, and the biopsy demonstrated RMS epithelioid type. Head magnetic resonance imaging (MRI) was performed and showed peripherally enhancing mass with central cystic/necrotic component. Computed tomography (CT) scan of the neck showed involvement of the cervical lymph nodes, while metastatic workup was negative for malignancy. As the biopsy showed a positive margin, the patient underwent secondary resection. Moreover, he received adjuvant chemotherapy and radiotherapy. There was no evidence of the disease, and no metastasis was detected in a follow-up of three years. We also performed a literature review of pediatric tongue RMSs to assess the clinical presentation, histopathology, diagnosis, and management.