From the Department of Pathology (TUL, NVL), Tufts Medical Center, Boston, Massachusetts, and Ophthalmology Arinella Williams LLC (JMW), Worcester, Massachusetts.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Tee U. Lang, Tufts Medical Center, 800 Washington St.. Dept of Pathology, Boston, MA 02111.
A myxoma is an uncommon, benign tumor of mesenchymal origin that has been identified in many sites of the body including the heart, skin, subcutaneous and aponeurotic tissues.1 Myxomatous lesions involving ophthalmic structures are rare but they have been described in the conjunctiva, orbit and eyelid.2 This is a case of limbal corneal myxoma in a middle-aged woman.
A 56-year-old woman referred to the ophthalmic service for evaluation of a lesion on her right cornea. There was no history of trauma or infection to the eye and there was no history of previous eye surgery or topical medications to the eye. Significant clinical history includes asthma and right breast carcinoma. Ophthalmic examination revealed a raised lesion with gelatinous appearance present at the nasal corneal limbus (Fig. 1). The lesion extended to approximately 3 mm onto the cornea. Clinically, the lesion was suspicious for squamous intraepithelial neoplasia and it was excised with cryotherapy. Paraffin-embedded, formalin-fixed, hematoxylin and eosin revealed a fragment of corneal tissue with squamous epithelium and Bowman’s layer that was focally disrupted (Fig. 2). There was no evidence of squamous carcinoma in situ. The underlying stroma was replaced by a myxoid matrix with scattered elongated spindle cells with no atypia (Fig. 2). Mitotic figures were not seen. No scar tissue was seen adjacent to this area. The myxoid matrix showed positive staining with Alcian blue pH 2.5 and was negative for elastic and periodic acid-schiff PAS stain. The findings were consistent with a corneal myxoma. At followup 1 and 3 months later, there was no clinical evidence of recurrence.
Figure 1. Raised Lesion with Myxoid Appearance near Nasal Limbus
Figure 2. Cornea with Stroma Replaced by a Myxoid Matrix with Scattered Elongated Spindle Cells with No Atypia (confirmed on Alcian Blue pH 2.5)
Elevated mass lesions of the cornea are relatively rare and the clinical differential diagnoses include Salzmann’s nodular degeneration, subepithelial amyloid deposits, and corneal keloid, primary or secondary tumors of the cornea.3 In our case, the clinical impression suggests a diagnosis of squamous cell carcinoma. The distinction between carcinoma and myxoma may prove difficult on clinical ground but each of these entities has a characteristic histologic appearance showing corneal stroma replaced by a myxoid matrix with scattered elongated spindle cells that have positive staining with Alcian blue pH 2.5.
Myxomas, in general, are well-circumscribed, smooth, translucent fleshy lesions that occur in adults with no sex predilection and a median age of 50 years (range 18 to 76 years) at the time of diagnosis.1 These lesions are slow growing and painless.3 It is also interesting that corneal myxoma has a predilection to form in the subepithelial anterior stroma.4
The origin of corneal myxoma has been suggested to arise from keratocytes due to their ultrastructural similarity to fibroblasts and the location of the tumor within the corneal stroma.3,4 Corneal myxoma is also considered a secondary reactive process.
In the case report by Perez Grossman et al., myxoma formation may require the interruption of Bowman’s layer and proximity of the scar to the epithelium.5 This suggestion has been observed in all, with exception of two, previously reported cases of corneal myxoma.3,4 The other reported cases of cornea myxoma had a preceding underlying pathology of the cornea, such as a corneal ulcer,5,6 keratitis with staplyloma,7 keratoconus,8 bullous keratopathy9 and repeated phototherapeutic keratectomies.10 The treatment of choice for corneal myxoma is local excision with free margins.1 Although they can recur with positive margin, in general, corneal myxoma is a benign lesion and it does not have metastatic potential.
Due to the rarity of the disease, our case highlights the importance of differentiating corneal myxoma, a benign tumor of mesenchymal origin, from other soft tissue tumors with myxomatous appearance.
- Pe’er J, Hidayat AA. Myxomas of the Conjunctiva: Am J Ophthalmol. 1986Jul15;102(1):80–86.
- Horie Y, Ikawa S, Okamoto I, Nagata M, Tamai A. Myxoma of the conjunctiva: a case report and a review of the literature. Jpn J Ophthalmol. 1995;39:77–82
- Lo GG, Biswas J, Rao NA, Font RL. Corneal myxoma. Case report and review of the literature. Cornea. 1990;9:174–178
- Hansen LH, Prause JU, Ehlers N, Heegaard S. Primary corneal myxoma. Acta ophthalmol Scand. 2004;82:224–227 doi:10.1111/j.1600-0420.2004.00249.x [CrossRef]
- Perez-Grossman RA, Mesias LA, Contreras F, Spencer WH. Solitary corneal myxoma. Cornea. 1997;16:498–500
- Bussy L. Lemyxome de la cornee. Arch Ophtalmol (Paris). 1925; 42:33–38.
- Mitvalsky J. Sur les myxomes de la cornee. Arch Ophtalmol (Paris). 1894;39:77–82.
- Leger F, Sawan B, Mortemousque B, Williamson W, Vital C. Corneal myxoma associated with keratoconus and Down’s syndrome. Cornea. 2000;19:561–563 doi:10.1097/00003226-200007000-00032 [CrossRef]
- Wollensak G, Green WR, Seiler T. Corneal myxoma. Jpn J Ophthalmol. 2002;46:193–197 doi:10.1016/S0021-5155(01)00493-2 [CrossRef]
- Robinson JW, Brownstein S, Mintsioulis G. Corneal myxoma arising in a patient with repeated phototherapeutic keratectomies. Cornea. 2006;25:1111–1114 doi:10.1097/01.ico.0000225710.54888.a1 [CrossRef]