Document Type

Article

Date of Publication

1-1-2023

Publication Title

Ophthalmic plastic and reconstructive surgery

First Page

e17

Last Page

e20

Abstract

A 3-year-old boy developed proptosis over 3 weeks. CT and MRI disclosed a 3.2 × 1.9 cm soft-tissue mass of the right extraconal and intraconal orbit with sphenoid bone erosion. After debulking through an upper eyelid crease incision, the tumor was diagnosed as a spindle cell/sclerosing rhabdomyosarcoma. DNA sequencing was negative for an L122R mutation in MyoD1 . Spindle cell/sclerosing rhabdomyosarcoma is an uncommon variant of this neoplasm, and only 2 patients with orbital tumors have been reported in 2 case series. Spindle cell/sclerosing rhabdomyosarcomas confined to the orbit are considered to have an excellent prognosis when treated with chemotherapy and radiation therapy. Diagnosis and treatment planning rely on histology, immunohistochemistry, and molecular analysis.

DOI

10.1097/IOP.0000000000002252

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