Orbital giant cell fibroblastoma

Abstract

BACKGROUND This report describes the clinical and morphologic (light and electron microscopic and immunohistochemical) features of orbital giant cell fibroblastoma which, to the best of our knowledge, has not been reported before in the ophthalmological literature. METHODS Clinical symptoms, computed tomography (CT), and magnetic resonance (MR) features, light and electron microscopic and immunohistochemical findings were reviewed in a 65-year-old woman who developed an orbital giant cell fibroblastoma. RESULTS The patient presented with a large, infiltrating, intra- and periorbital mass, causing severe proptosis, ocular displacement, and visual loss on the left side. CT and MR imaging demonstrated a large, irregular tissue density in the orbit leading to displacement of the globe anteriorly and inferolaterally. With T 1 -weighted MR images, the lesion was isointense to the gray matter of the brain and revealed marked enhancement with Gd-DTPA. Histopathologically, the tumor consisted of a mixture of spindle and multinucleated giant cells scattered within a myxomatous stroma. CONCLUSION Giant cell fibroblastoma, a benign mesenchymal tumor of infancy, is rarely encountered within the orbital region. This lesion is considered to be a distinct, non-metastasizing tumor, but its clinical management may be very difficult, as in our case, because of its invasive nature and potential for recurrence.