Extensive alveolar-type paranasal sinus and orbit rhabdomyosarcoma with intracranial invasion treated successfully

Abstract

We report a case of extensive paranasal sinus and orbit rhabdomyosarcoma (RMS) with intra-cranial invasion treated successfully with chemotherapy and radiotherapy. A 13-years-old male patient complained of painless and progressive proptosis of his left eye for two weeks. Ocular examination showed elevated intraocular pressure, limited extraocular movement, proptosis, and conjunctival ciliary injection in the left eye. Brain CT and MRI demonstrated a large enhancing soft tissue mass lesion with bone destruction involving left ethmoid sinus, nasal cavity, maxillary sinus, and orbital cavity with crossing of the midline to the right ethmoid sinus, nasal cavity, and intra-cranial invasion across the frontal base. The pathology of tumor biopsy revealed rhabdomyosarcoma, alveolar type. Systemic survey showed no evidence of distant metastasis. Then, the patient received combined radiochemotherapy with Taiwan Pediatric Oncology Group Rhabdomyosarcoma 2007 High-risk Treatment Protocol. No light perception in his left eye with optic disc atrophy was noted at the beginning of radiotherapy. After 44 weeks of combined radiochemotherapy, the tumor regressed, and no recurrence has been noted until now. In young patients with sudden-onset proptosis, RMS should be considered, and early diagnosis is crucial due to more effective prognosis with current radiochemotherapy protocol.