A Rare Presentation of Retinoblastoma as a Fungating Orbital Mass: A Case Report
Journal Title: Journal of Academic Research in Medicine - Year 2020, Vol 10, Issue 3
Abstract
Retinoblastoma (RBL) arises from the precursor cells of the retinal neuroepithelium. It is the most common primary malignant intraocular tumor in children. Primary orbital RBL is an extraocular disease detected clinically or radiologically at the time of diagnosis. It is a direct metastasis of an intraocular RBL. It very rarely presents as an exuberant fungating orbital mass, like in the present case, and is found in more advanced, untreated cases. Magnetic resonance imaging (MRI) is the preferred imaging modality for the evaluation of the tumor, vital orbital structures such as the optic nerve, and intracranial involvements. Computed tomography (CT) is useful in the evaluation of adjacent bony structures and calcifications. Herein, we report the case of a 3-year-old boy who presented to our clinic with a large, rapidly growing cauliflower-like mass protruding out of the left orbit and invading the surrounding soft tissues. MRI showed a10×8×7.5 cm3 infiltrative heterogeneously enhancing left orbital mass with some necrotic and hemorrhagic components. Metastatic leptomeningeal enhancements were widespread in the intracranial region. CT revealed erosive destructive changes at the lateral orbital wall. Following left orbital enucleation, the diagnosis of RBL was made by histopathological examination, which showed sporadic Flexner-Wintersteiner type rosette formations. The patient underwent surgery and thereafter received radiotherapy and adjuvant chemotherapy. No recurrence was observed after 5 years. Although rare, orbital extension of RBL is one of the major contributors to mortality. Early diagnosis and detailed radiological evaluation are necessary to establish intracranial involvement and distant metastasis at the time of diagnosis.
Authors and Affiliations
Kenan Kıbıcı, Berrin Erok, Onat Akın
Keywords
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- EP ID EP691773
- DOI 10.4274/jarem.galenos.2020.3630
- Views 104
- Downloads 0
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