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Plexiform Neurofibroma Involving the Lacrimal Gland

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BACKGROUND: To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid.

METHODS: A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed.

RESULTS: Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch.

CONCLUSION: This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.

Original languageEnglish
JournalCase Reports in Ophthalmology
Volume8
Issue number1
Pages (from-to)67-72
Number of pages6
ISSN1663-2699
DOIs
Publication statusPublished - 17 Feb 2017

    Research areas

  • Journal Article

ID: 52765146