A rare presentation of retinoblastoma as a fungating orbital mass: A case report

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dc.contributor.authorKıbıcı, Kenan
dc.contributor.authorErok, Berrin
dc.contributor.authorAkın, Onat
dc.date.accessioned2021-05-15T11:33:22Z
dc.date.available2021-05-15T11:33:22Z
dc.date.issued2020
dc.departmentTıp Fakültesi, Nöroşirürji Kliniğien_US
dc.descriptionerok, berrin/0000-0001-8036-547X
dc.description.abstractRetinoblastoma (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 a10x8x7.5 cm(3) 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.en_US
dc.identifier.doi10.4274/jarem.galenos.2020.3630
dc.identifier.endpage302en_US
dc.identifier.issn2146-6505
dc.identifier.issn2147-1894
dc.identifier.issue3en_US
dc.identifier.startpage298en_US
dc.identifier.trdizinid417176
dc.identifier.urihttps://doi.org/10.4274/jarem.galenos.2020.3630
dc.identifier.urihttps://hdl.handle.net/20.500.12939/142
dc.identifier.volume10en_US
dc.identifier.wosWOS:000604280800017
dc.identifier.wosqualityN/A
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakTR-Dizin
dc.institutionauthorKıbıcı, Kenan
dc.language.isoen
dc.publisherGalenos Yayinciliken_US
dc.relation.ispartofJournal of Academic Research in Medicine-Jarem
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectPrimary Orbital Retinoblastomaen_US
dc.subjectFlexner-Wintersteiner Rosetteen_US
dc.subjectMagnetic Resonance Imaging (MRI)en_US
dc.subjectLeptomeningeal Metastasisen_US
dc.titleA rare presentation of retinoblastoma as a fungating orbital mass: A case report
dc.typeArticle

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