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

Author:

Shrestha Tina1,Mainali Sumina2ORCID,Poudel Sandhya1,K.C Narendra2,Dulal Aliza2

Affiliation:

1. Department of Ophthalmology, Dhulikhel hospital

2. Kathmandu University School of Medical Sciences, Dhulikhel, Kavre

Abstract

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 3.2×2.7×5.4 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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

General Medicine,Surgery

Reference9 articles.

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2. Pediatric primary orbital rhabdomyosarcoma;Thien;J Pediatr Surg Case Reports,2020

3. Orbital rhabdomyosarcoma in the pediatric population: a prospective, observational study from mid-west Nepal;Bastola;Acad J Heal Sci Med Balear ISSN-e 2255-0560,2022

4. Aggressive orbital rhabdomyosarcoma in adulthood: a case report in a public hospital in Damascus, Syria;Ahmad;Avicenna J Med [Internet],2018

5. Primary orbital rhabdomyosarcoma with skeletal muscle metastasis;Das;Oman J Ophthalmol [Internet],2010

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