A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit.

Lee, Myoung Ok; Jeon, Chan; Suh, Yeon Lim; Woo, Kyung In; Kim, Yoon Duck

doi:2009.50.9.1409

J Korean Ophthalmol Soc > Volume 50(9); 2009 > Article

Case Report

Journal of the Korean Ophthalmological Society 2009;50(9):1409-1413.
DOI: https://doi.org/10.3341/jkos.2009.50.9.1409 Published online September 15, 2009.

A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit.

Myoung Ok Lee, Chan Jeon, Yeon Lim Suh, Kyung In Woo, Yoon Duck Kim

¹Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. ydkimoph@skku.edu
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.

안와 내에 발생한 악성말초신경초종양 1예

이명옥¹ㆍ전찬²ㆍ서연림³ㆍ우경인¹ㆍ김윤덕¹

Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine¹, Seoul, Korea / Department of Ophthalmology, Hallym University College of Medicine, Hallym University Sacred Heart Hospital^{2/sup>, Seoul, Korea / Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine³, Seoul, Korea}

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.

Key Words: Malignant peripheral nerve sheath tumor;Orbit