J Korean Ophthalmol Soc.  2009 Sep;50(9):1409-1413. 10.3341/jkos.2009.50.9.1409.

A Case of Malignant Peripheral Nerve Sheath Tumor of the Orbit

• Lee MO
• Jeon C
• Suh YL
• Woo KI
• Kim YD

• KMID: 2212626
• DOI: http://doi.org/10.3341/jkos.2009.50.9.1409

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.

Keyword

Malignant peripheral nerve sheath tumor; Orbit

MeSH Terms

Aged
Biopsy
Diagnosis, Differential
Eyelids
Humans
Orbit
Peripheral Nerves

Figure

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  Figure 1. Clinical photograph showing edema and proptosis of the right eye.

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  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.

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  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.

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  Figure 4. Low-power magnification of the tumor shows spindle cells arranged in fascicular pattern with necrotic foci (hematoxylin and eosin, ×100).

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  Figure 5. High-power magnification reveals mitotic figures and nuclear pleomorphism (hematoxylin and eosin, ×200).

Reference

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