RESUMEN
Focal reactive nodular gliosis (FRNG) is an extremely rare benign retinal reactive astrocytic tumor that results from the proliferation of well-differentiated glial cells secondary to a variety of retinal conditions. We describe a case of this tumor in a 64-year-old male in association with a chorioretinal scar he has had since childhood. The symptom was sudden painful vision loss. In the clinical examination, iris rubeosis, posterior synechiae, cataract, vitreous haze and a suspected fundus mass were showed. B-scan ultrasonography demonstrated a retinal mass consistent with choroidal melanoma. The magnetic resonance imaging (MRI) showed a well-circumscribed mass with T1 hyperintensity and T2 hypointensity. Enucleation was performed and histopathologic and immunohistochemical studies confirmed the diagnosis of FRNG.
RESUMEN
ABSTRACT Combined hamartoma of the retina and retinal pigment epithelium is a rare, benign intraocular tumor. Hamartoma of the retina and retinal pigment epithelium has been described in the literature as a condition presenting with variable retinal damage, ranging from partial epiretinal involvement to complete distortion of the retinal layers and retinal pigment epithelium. We report the case of an 8-year-old girl presenting with longstanding strabismus who was diagnosed with Hamartoma of the retina and retinal pigment epithelium based on multimodal imaging assessment. We explored the particular imaging findings from studies using spectral-domain optical coherence tomography, fundus autofluorescence, optical coherence tomography angiography, and fluorescein angiography.
RESUMO O hamartoma combinado de retina e epitélio pigmentar da retina consiste em um tumor intraocular raro com comportamento benigno. O hamartoma combinado de retina e epitélio pigmentar da retina foi descrito na literatura apresentando dano retiniano variável, desde o envolvimento epirretiniano parcial até distorção completa das camadas retinianas e do epitélio pigmentar da retina. Relatamos o caso de uma menina de 8 anos com estrabismo de longa data que foi diagnosticada com hamartoma combinado de retina e epitélio pigmentar da retina, com base na avaliação de imagem multimodal. Exploramos os achados de imagem específicos de estudos usando tomografia de coerência óptica de domínio espectral, autofluorescência, angiografia por tomografia de coerência óptica e angiografia fluorescente.
RESUMEN
OBJECTIVE: To present the characteristics and treatment outcomes of patients with retinoblastoma. METHODS: A retrospective case series was conducted to review the records of all new patients diagnosed with retinoblastoma between 2003 and 2005. Eyes with early disease, or advanced disease with potential vision were treated with chemotherapy (carboplatin and etoposide) in conjunction with early local therapy (laser or cryo). Radiotherapy was used in cases where the disease did not respond to the above protocols or in recurrent cases. Eyes in the late stage of disease with no potential vision in the initial examination or eyes and where conservative treatment had failed were enucleated. RESULTS: In total, we reviewed 28 new cases of retinoblastoma, 15 of which were unilateral and 13 of which were bilateral (46 percent). These data correspond to a mean of 9.3 new cases per year (0.77 case/ month). The mean age at diagnosis was 33.8 months for unilateral cases, and 19.15 months for bilateral cases (p=0.015). Leucocoria was the major presenting symptom (75 percent). All but one patient with unilateral disease had the affected eye enucleated due to advanced disease (mean follow-up: 39.91 months). Among the 13 bilateral cases, 13 eyes (50 percent) were enucleated, 11 eyes (42.4 percent) were saved with chemotherapy in conjunction with local therapy and 2 eyes (7.6 percent) were saved using external beam radiotherapy (mean follow-up: 41.91 months). In unilateral and bilateral disease, pathology data revealed choroid involvement in 50 percent and 30 percent, respectively, and optic nerve invasion in 92 percent and 50 percent, respectively. CONCLUSION: In this population, retinoblastoma was diagnosed too late and most eyes were consequently enucleated. In cases with bilateral disease, half of the eyes were preserved.