Spindle Cell/Sclerosing Rhabdomyosarcoma of the Orbit

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 rhabdomyosarc...

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Bibliographic Details
Published inOphthalmic plastic and reconstructive surgery Vol. 39; no. 1; p. e17
Main Author Proia, Alan D
Format Journal Article
LanguageEnglish
Published United States 01.01.2023
Subjects
Child, Preschool
Humans
Male
Mutation
Orbit - diagnostic imaging
Orbit - pathology
Orbital Neoplasms - diagnosis
Orbital Neoplasms - therapy
Rhabdomyosarcoma - diagnosis
Rhabdomyosarcoma - therapy
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Summary: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.
ISSN:1537-2677
DOI:10.1097/IOP.0000000000002252