학술논문

Orbital rhabdomyosarcoma: An atypical presentation
Document Type
article
Source
Kerala Journal of Ophthalmology, Vol 33, Iss 1, Pp 71-74 (2021)
Subject
embryonal rhabdomyosarcoma
immunohistochemistry
lid swelling
orbital rhabdomyosarcoma
Ophthalmology
RE1-994
Language
English
ISSN
0976-6677
Abstract
Orbital Rhabdomyosarcoma is a lethal malignancy of childhood. It commonly presents in the first decade of life as a rapidly progressive proptosis that can mimic orbital inflammatory disorders. We present a case of a 13-year-old boy who presented with a diffuse right upper lid swelling progressing over 10 days. The detailed evaluation revealed an immobile, firm, and eyelid mass adhering to underlying tissues, not adherent to the skin. The provisional diagnosis of an orbital inflammation was performed. Neuroimaging suggested a preseptal cystic lesion. Intraoperatively, a solid bosselated mass arising from orbicularis oculi, extending into the anterior orbit was excised in toto. Histopathology revealed Stage-1 embryonal rhabdomyosarcoma, confirmed on immunohistochemistry. Postoperatively, the patient underwent systemic chemotherapy. The patient was tumor-free at 2 years follow-up.