PMC5301085_01
A Initial clinical photograph of the patient (2 years of age) showing ptosis of the right eyelid and a solid mass located on the right upper lid. B Magnetic resonance imaging scan of the cerebrum and orbit revealing a solid tumor located extraconally at the site of the right lacrimal gland (arrow). C Markedly enlarged nerve segments and thickened nerve bundles (*) within the lacrimal gland tissue (arrow). Hematoxylin and eosin section. Scale bar = 1: 200 microm. D Higher magnification showing bundles of nerve fibers arranged in a concentric manner with areas of myxoid changes. Schwann cells and fibroblasts are seen. Hematoxylin and eosin section. Scale bar = 1: 200 microm. Normal lacrimal gland tissue (arrow) is seen between the nerve fiber bundles (*).
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F1
There was a characteristic s-shaped deformity of the right upper eyelid typical of NF1, and the eyelid was soft on palpation (Fig. 1A). Magnetic resonance imaging (MRI) scans of the cerebrum and the orbit were performed and showed exophthalmus and a solid tumor, 1 cm at the largest axis, located extraconally at the site of the lacrimal gland and highly suggestive of a plexiform neurofibroma or a lacrimal gland tumor (Fig. 1B).