A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report

Introduction and Importance:Orbital rhabdomyosarcoma (RMS) is a highly malignant, mesenchymal orbital tumor of childhood with a predilection in children less than 20 years of age. It presents as a space-occupying lesion in the orbit, most commonly over the superior nasal quadrant of the orbit. The patient usually presents with rapid onset unilateral proptosis and eyelid edema. Case Ppresentation:In this article, a 14-year-old male presented with rapidly progressive swelling of the right orbit. On ocular examination, there was nonaxial inferolateral proptosis of the right eye. Computed tomography revealed a large soft tissue density tissue lesion in the right nasal cavity and meati measuring at least 3.2x2.7x5.4 cm in size with the erosion of the right orbit along with extension of the lesion in the extraconal compartment of the orbit. An MRI of the brain with contrast showed a heterogeneously enhancing altered signal intensity lesion. Debulking was planned, and a biopsy of the mass was sent that gave an impression of alveolar RMS. He also received radiotherapy and chemotherapy at one of the cancer hospitals in Nepal. Postsurgical follow-up showed gradual improvement in the visual acuity of the right eye. No evidence of metastasis and recurrence was found upon subsequent follow-up. Conclusion:Thus, early diagnosis and prompt treatment is most for a favorable prognosis in the case of RMS. The main aim of this article was to briefly overview a rare case of RMS, its clinical presentation, diagnosis, treatment modalities, and its prognosis.