Published Date: April 28, 2023Full Text Article
A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
Authors: Tina Shrestha, Sumina Mainali, Sandhya Poudel, Narendra K C, Aliza Dulal
Ann Med Surg (Lond). 2023 Apr 10;85(4):1247-1253. doi: 10.1097/MS9.0000000000000501. eCollection 2023 Apr.
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 3.2×2.7×5.4 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.