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

Authors
  • Shrestha, Tina1
  • Mainali, Sumina2
  • Poudel, Sandhya1
  • K.C, Narendra2
  • Dulal, Aliza2
  • 1 Department of Ophthalmology, Dhulikhel hospital
  • 2 Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
Type
Published Article
Journal
Annals of Medicine and Surgery
Publisher
Elsevier BV
Publication Date
Apr 10, 2023
Volume
85
Issue
4
Pages
1247–1253
Identifiers
DOI: 10.1097/MS9.0000000000000501
PMID: 37113866
PMCID: PMC10129160
Source
PubMed Central
Keywords
Disciplines
  • Case Reports
License
Unknown

Abstract

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.

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