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Updated: Aug 20, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Malignant peripheral nerve sheath tumour metastatic to the orbit
Dinesh Selva1, Garry Davis, Tom Dodd
1Department of Ophthalmology, University of British Columbia and the Vancouver General Hospital, Vancouver, Canada.
Purpose:
To present a case of malignant peripheral nerve sheath tumour (MPNST) metastatic to the orbit.
Method/Results:
The authors describe a 29-year-old woman with disseminated MPNST who presented with acute right orbital inflammation. Computerized tomography revealed a superolateral mass with a central radiolucent area. At biopsy, the mass was primarily subperiosteal and histology showed a spindle cell, S100-positive MPNST consistent with the primary tumour.
Conclusion:
This is the second reported case of MPNST metastatic to the orbit and illustrates that an inflammatory presentation is possible in this aggressive malignancy.
