Introduction: Rhabdomyosarcoma is uncommon in adults. Adult and maxillary rhabomyosarcoma with direct orbital extension has been rarely reported. To our knowledge, there is no reported case about adult patient with alveolar maxillary rhabdomyosarcoma and orbital extension survived 1 year with intact ocular function.
Case presentation: A 21-year-old female presented with protrusion of the right eye and an obstructed nasal passage for the past month. Her symptoms were not relieved by oral antibiotic or irrigation. She was referred to our clinic. Computed tomography and magnetic resonance imaging showed a large homogenous well-enhanced mass with surrounding bony erosion and remodeling. The mass extended to the ipsilateral nasal cavity and orbit. Endoscopic biopsy of the nasal cavity confirmed alveolar rhabdomyosarcoma. The maxillary mass was excised using the Caldwell-Luc approach, and the orbital mass was excised using a transconjuctival and transcaruncular approach. A systemic work-up confirmed ipsilateral lymph node metastasis. The patient received 6 cycles of chemotherapy with vincristine, dactinomycin, and cyclophosphamide, as well as 5120 cGy radiotherapy. Her ocular function was intact 1 year after treatment, and magnetic resonance imaging showed complete regression of the tumor.
Conclusion: Rhabdomyosarcoma, which is usually an aggressive malignancy, should be considered in the differential diagnosis of a rapidly growing orbital mass. Aggressive treatment, including surgery, chemotherapy, and radiation therapy, can increase local remission rates and improve the prognosis.