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2, Gyeonggi, Korea Purpose: To report a case of orbital leiomyoma presenting with proptosis. Case summary: A 69‐year‐old woman presented with a 3‐month history of proptosis in her left eye. Intraocular pressure was 17 mmHg in her right eye and 23 mmHg in her left eye. There was a left hypotropia on upgaze. A fundus examination showed retinal folds in the superotemporal area in her left eye. Computed tomography revealed a 2.6 cm‐sized well‐defined enhancing solid mass in the superotemporal extraconal space of the left orbit, pushing her left eye forward. Lateral orbitotomy, tumor removal, and biopsy were performed. Pathological findings showed a fascicular pattern of benign spindle cells with mild cellular pleomorphism and hyaline degeneration, without mitotic figures. Immunohistochemical stain was positive with smooth muscle actin (SMA), which was compatible with orbital leiomyoma.

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.