Background: Fibrous histiocytoma (FH), a mesenchymal tumour, usually have an orbital presentation. Rarely, FH in both benign and malignant forms have been reported at limbus and conjunctiva. Present narrative review was conducted to determine demographic profile, clinical presentation and management options for this rare tumour.Methods: PubMed database was searched to identify articles presenting with fibrous histiocytoma of conjunctiva, cornea and limbus. Data was tabulated for age of presentation, sex, eye involved, area of involvement, if tumor was benign or malignant, management and recurrences.Results: Total of 35 articles were selected, which included 42 cases, of which 27 were benign and 15 malignant. 25 cases showed presence of limbal FH, 12 conjunctival, 4 caruncular and 1 corneal FH. Mean age of presentation was 30.9 years with male preponderance (females-10, males-17) in benign FH while malignant FH presented with mean age of 44.5 years and sex distribution of 7 females and 8 males. 4 cases of benign FH showed recurrence, which required re-excision and immunotherapy, while recurrence in malignant FH was 60%, where 4 needed exenteration, 3 enucleation and 1 expired due to metastasis.Conclusion: FH is one of the rare ocular tumours of eye presenting usually at the limbus and conjunctiva and rarely cornea. Histopathological examination along with immunostaining has a definite role in the diagnosis. Surgical excision with tumour free margins is the corner stone of treatment. Recurrences in benign varieties need re-excision or immunotherapy, while malignant cases need aggressive surgical options like exenteration or enucleation.
Keywords: Benign fibrous histiocytoma; Conjunctiva; Cornea; Fibrous histiocytoma; Malignant fibrous histiocytoma.