Primary corneal tumors are rare. In fact, corneal tumors secondary to the extension of a conjunctival tumor, in particular limbic, are more frequently observed (Shields and Shields 2019). Corneal tumors correspond to congenital or acquired lesions. Usually, to classify them, we use the classification of conjunctival tumors, according to the histological aspect, we distinguish between epithelial, melanocytic and mesenchymal tumors (Rao and Shields 2019). The latter include fibrous tumors which are extremely rare and benign.
A 7-month old female patient was referred to our department for huge reddish smooth non-growing corneal tumor in her right eye present since birth, she was a healthy term infant with no significant familial medical history, she was born of a non-consanguineous marriage, her vaccination was completed according to Morocco’s national immunization programme.
The right eye showed a huge elevated smooth, reddish-colored mass taking all the thickness of the cornea (Figure 1, 2). Few dilated, small caliber vessels were seen in the limbus; the conjunctiva seems normal. The left eye examination was unremarkable. The general examination showed no sign of congenital generalized fibromatosis.
The ocular echography highlights an enormous hyperechgenic tumor, the other ophtalmological structures were normal.
The orbito-cerebral MRI showed the corneal tumor measuring 15 length x 13mm width, with no contrast enhancement. No associated abnormalities were found (Figure 3).
The enucleation was performed after getting written consent from the parents.
The histopathological examination was remarkable:
In Figure 4 the corneal mucosa is covered by squamous epithelium with hyperkeratosis packed with abundant collagenous stroma and some spindle-shaped cells, no atypia and no mitotic figures were seen. Immunohistochemically, the cells stained negatively for CD-68, S-100.
In the light of the histopahology and immunochemical staining, a diagnosis of corneal fibroma was made.
Fibromas of the ocular surface are exceptionally rare. Conjunctival or epibulbar fibromas are speculated to arise from the Tenon’s fascia (Nair et al. 2018).
Fibroma is a benign neoplasm composed of fibrous connective tissue. At least three distinct forms of fibroma, characterised by dense, paucicellular collagen and benign behaviour, have been described as being present within the skin and subcutaneous tissue. Congenital fibrous tumors of cornea are likely attributed to undifferentiated hyperplasia of opaque corneal and scleral tissue of developmental origin (Hayat et al. 2010). They are very rare, especially when they are congenital and not associated with generalized fibromatosis.
In our case, the atypical presentation and the size of the tumor made a simple excision hard to achieve, also, the post-operative tissue defect would be difficult to completely cover; the enucleation was the best surgical choice to perform.
After 1 year follow up, no similar lesion appeared.
Corneal fibroma is a very exceptional tumor. Only few reports are described. We aim throughout our clinical case to contribute to the medical literature by reporting such atypical presentation.
Financial support and sponsorship
Conflict of Interest
We confirm and deny any conflicts of interest existing.