Synovial sarcoma (SS) is the most common nonrhabdomyosarcomatous soft tissue sarcoma in childhood, but the head-neck site accounts for less than 5% of cases. The authors report a 10-year-old boy with SYT-SSX1 positive left parapharyngeal SS, resistant to front-line VAIA chemotherapy, who obtained a good partial response by salvage regimen (I(3)VE + CEV + I(3)VE) and local radiotherapy, so a complete surgical resection could be performed. The complete remission was subsequently consolidated by ablative high-dose chemotherapy, followed by autologous stem cell reinfusion. The child remains in complete remission at 36 months after completion of treatment.

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