Corneal Myofibroma (Keloid) in a Young Patient with Neurofibromatosis Type 2

Abstract

We present a 27-year-old male patient with neurofibromatosis type 2 (NF2), facial palsy, and lagophthalmos following acoustic neuroma removal and an impressing vascularized corneal tumor, which was excised. Histology showed a fibrous tumor with small vessels, and immunohistochemistry was positive for vimentin and negative for smooth muscle actin, S100, and GFAP. We assume a corneal myofibroma (keloid), which in this case rather represents a reactive lesion. This entity has not been described before in NF2 or in facial palsy-associated lagophthalmos in general.

Keywords: Corneal myofibroma; Keloid; Neurofibromatosis type 2.

Fig. 1

A whitish tumor on the cornea of the left eye with multiple vessel tufts within the tumor stroma of a 27-year-old male patient with known neurofibromatosis type 2.

Fig. 2

Histology showing hyperplastic, non-keratinizing corneal epithelium with metaplasia towards keratinizing epithelium and dense vascularized fibromatous tissue (hematoxylin-eosin, original magnification ×100).

Fig. 3

Upper half: low-power magnification of the specimen with the fibrous tissue highlighted by bluish positivity for Masson trichrome (original magnification ×40). Lower half: high-power magnification of the tumor stroma with small blood vessels of the area highlighted by the square (Masson trichrome, original magnification ×200).

Fig. 4

Mainly negative staining of tumor cells for smooth muscle actin, whilst adjacent vessels were positive (arrow).