Objective: This report describes an exceedingly rare case of adult embryonal rhabdomyosarcoma arising in the head and neck, which was initially diagnosed as a primary unknown cancer.
Method: The patient underwent a radical neck dissection with the total excision of the tumor, which was diagnosed as embryonal rhabdomyosarcoma.
Result: The patient received intensive adjuvant chemotherapy, and is still alive with no signs of recurrence or metastasis.
Conclusion: This case report demonstrates not only the rarity of this neoplasm but the importance of the differential diagnosis for planning the therapeutic strategy.
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