A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit.
10.3341/jkos.2009.50.9.1409
- Author:
Myoung Ok LEE
1
;
Chan JEON
;
Yeon Lim SUH
;
Kyung In WOO
;
Yoon Duck KIM
Author Information
1. Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. ydkimoph@skku.edu
- Publication Type:Case Report
- Keywords:
Malignant peripheral nerve sheath tumor;
Orbit
- MeSH:
Aged;
Biopsy;
Diagnosis, Differential;
Eyelids;
Humans;
Orbit;
Peripheral Nerves
- From:Journal of the Korean Ophthalmological Society
2009;50(9):1409-1413
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
PURPOSE: To present a case of malignant peripheral nerve sheath tumor (MPNST) in the orbit. CASE SUMMARY: A 66-year-old man was evaluated for swelling of the left upper eyelid without pain that developed 2 months earlier. ACT scan and MRI of the orbit showed a large superior orbital mass with adjacent bony erosion, which had a central necrotic area. Incisional biopsy of the orbital mass was performed through a sub-brow incision. Histopathologic examination revealed a hypercellular tumor composed of spindle-shaped pleomorphic cells arranged in a fascicular pattern with necrotic foci. High-power magnification showed mitotic figures and nuclear pleomorphism. Subsequently, a malignant peripheral nerve sheath tumor was diagnosed. CONCLUSIONS: The possibility of malignant peripheral nerve sheath tumor should be considered in the differential diagnosis of an orbital mass which grows rapidly and shows bone invasion. Salvage surgery should be performed promptly to increase the likelihood of long-term survival.
