Journal List > J Korean Ophthalmol Soc > v.50(9) > 1008371

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Lee, Jeon, Suh, Woo, and Kim: A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit
Original Article

J Korean Ophthalmol Soc 2009;50(9):1409-1413.

Published online: 7 September 2009

DOI: https://doi.org/10.3341/jkos.2009.50.9.1409

A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit

Myoung-Ok Lee, MD1, Chan Jeon, MD2, Yeon-Lim Suh, MD, PhD3, Kyung-In Woo, MD, PhD1, Yoon-Duck Kim, MD, PhD1

1Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

2Department of Ophthalmology, Hallym University College of Medicine, Hallym University Sacred Heart Hospital, Seoul, Korea

3Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Address reprint requests to Yoon-Duck Kim, MD, PhD Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine #50 Ilwon-dong, Ganam-gu, Seoul 135-710, Korea Tel: 82-2-3410-3561, Fax: 82-2-3410-0074, E-mail: ydkimoph@skku.edu

Received 6 January 2009       Accepted 26 May 2009

Copyright © 2009 Korean Ophthalmological Society

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Malignant peripheral nerve sheath tumor, Orbit

References

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Figure 1.
Clinical photograph showing edema and proptosis of the right eye.
jkos-50-1409f1.tif
Figure 2.
(A) Axial CT reveals a large superior orbital mass with adjacent bony erosion. (B) Coronal CT depicts invasion of the superior muscle by the mass with destruction of the superior orbital wall.
jkos-50-1409f2.tif
Figure 3.
(A) T1-weighted precontrast axial image demonstrating a well-defined mass in the right orbit with central necrosis. (B) T2-weighted precontrast axial image showing high signal intensity in the central necrotic area. (C, D, E) T1-weighted postcontrast images demonstrating significant homogenous peripheral enhancement and invasion into the dura mater without intracranial extension.
jkos-50-1409f3.tif
Figure 4.
Low-power magnification of the tumor shows spindle cells arranged in fascicular pattern with necrotic foci (hematoxylin and eosin, ×100).
jkos-50-1409f4.tif
Figure 5.
High-power magnification reveals mitotic figures and nuclear pleomorphism (hematoxylin and eosin, ×200).
jkos-50-1409f5.tif
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