A Case of Intratemporal Rhabdomyosarcoma in a Child Presenting with VIIth, IXth, and Xth Cranial Nerve Paralysis.

OBJECTIVE:Rhabdomyosarcoma is the most common soft tissue tumor in children, with average age of onset being 5 years, and approximately 70% cases diagnosed below 10 years of age. It accounts for 37% of primary head and neck malignancies in children. Chemotherapy with surgery, and radiation is selected as the primary treatment. We report a rare case of rhabdomyosarcoma in the temporal bone presenting with glossopharyngeal and vagus nerve paralysis as well as facial palsy. CASE REPORT:The patient was a 6-year-old boy, and his initial symptom was dizziness followed by facial palsy and hoarseness. Although a severe type of otitis media was suspected in the first clinic, CT and MRI showed a temporal bone tumor with parameningeal extension. Biopsy with cortical mastoidectomy revealed an embryonal-type rhabdomyosarcoma. Pretreatment re-excision was abandoned because of parameningeal involvement. The tumor disappeared after a series of chemotherapy, however, meningeal dissemination occurred, and he eventually died even after an additional administration of anti-cancer agents and intensive modulated radiation therapy. CONCLUSION:In the case of facial palsy concomitant with other cranial nerve paralysis, care must be taken into neoplastic origin. Early image diagnosis may offer a chance of complete resection in addition to chemoradiotherapy.