Ethmoidal–nasal alveolar rhabdomyosarcoma in an adolescent: The contribution of radiotherapy in a high-risk case

Alveolar rhabdomyosarcoma (ARMS), a histological subtype associated with a poorer prognosis, represents an aggressive form of the disease. In the ethmoidal–nasal region, the proximity of critical structures such as the orbit and the skull base presents significant diagnostic and therapeutic challenges.

We report the case of a 15-year-old adolescent presenting with sudden-onset right exophthalmos secondary to an alveolar RMS with orbital, sinus, and basifrontal extension. A multimodal treatment approach was initiated, including ifosfamide–vincristine–actinomycin (IVA) chemotherapy followed by intensity-modulated radiation therapy (IMRT) delivered concomitantly with chemotherapy. The treatment was overall well tolerated. Immediate post-treatment imaging showed slight tumor regression, and at 18 months a marked regression of both the tumor and cervical lymphadenopathies was observed after maintenance therapy combining actinomycin, cyclophosphamide, and vinorelbine.

This case highlights the importance of a multimodal therapeutic strategy and multidisciplinary management in high-risk ethmoidal–nasal RMS. Long-term surveillance remains essential to optimize prognosis and allow early detection of recurrence.