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ABSTRACT Purpose: This study aimed to analyze the association between magnetic resonance imaging apparent diffusion coefficient map value and histopathological differentiation in patients who underwent eye enucleation due to retinoblastomas. Methods: An observational chart review study of patients with retinoblastoma that had histopathology of the lesion and orbit magnetic resonance imaging with apparent diffusion coefficient analysis at Hospital de Clínicas de Porto Alegre between November 2013 and November 2016 was performed. The histopathology was reviewed after enucleation. To analyze the difference in apparent diffusion coefficient values between the two major histopathological prognostic groups, Student's t-test was used for the two groups. All statistical analyses were performed using SPSS version 19.0 for Microsoft Windows (SPSS, Inc., Chicago, IL, USA). Our institutional review board approved this retrospective study without obtaining informed consent. Results: Thirteen children were evaluated, and only eight underwent eye enucleation and were included in the analysis. The others were treated with photocoagulation, embolization, radiotherapy, and chemotherapy and were excluded due to the lack of histopathological results. When compared with histopathology, magnetic resonance imaging demonstrated 100% accuracy in retinoblastoma diagnosis. Optic nerve invasion detection on magnetic resonance imaging showed a 66.6% sensitivity and 80.0% specificity. Positive and negative predictive values were 66.6% and 80.0%, respectively, with an accuracy of 75%. In addition, the mean apparent diffusion coefficient of the eight eyes was 0.615 × 103 mm2/s. The mean apparent diffusion coefficient value of poorly or undifferentiated retinoblastoma and differentiated tumors were 0.520 × 103 mm2/s and 0.774 × 103 mm2/s, respectively. Conclusion: This study revealed that magnetic resonance imaging is useful in the diagnosis of retinoblastoma and detection of optic nerve infiltration, with a sensitivity of 66.6% and specificity of 80%. Our results also showed lower apparent diffusion coefficient values in poorly differentiated retinoblastomas with a mean of 0.520 × 103 mm2/s, whereas in well and moderately differentiated, the mean was 0.774 × 103 mm2/s.

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ABSTRACT Objective: To assess the deaths caused by eye cancer from 2010 to 2019 in Brazil. Methods: Data were selected from SUS' Computer Department platform at the Ministry of Health, including death certificates, from 2010 to 2019, from all Brazilian states and the Federal District, filtering the codes C69.0 to C69.9 as the cause of death, according to the International Classification of Diseases, Tenth Revision. Results: There were 1,859 deaths from malignant neoplasm of eye and adnexa (C69), in Brazil, from 2010 to 2019, affecting 1,062 (57.1%) men. The site of neoplasm was unspecified (C69.9) in 719 cases, representing the most frequent etiology in the C69 group (38.67%). The malignant neoplasm of the orbit (C69.6) was the second most common cause of death (22.59%), followed by malignant neoplasm of retina (C69.2) (14.73%). Conclusion: The number of deaths due to malignant neoplasm of eye and adnexa slightly increased through the years of 2010 to 2019, in Brazil.

RESUMO Objetivo: Avaliar os óbitos causados por câncer ocular durante os anos de 2010 a 2019 no Brasil. Métodos: Os dados foram selecionados na plataforma do Departamento de Informática do SUS do Ministério da Saúde, incluindo declarações de óbito, durante os anos de 2010 a 2019, de todos os estados brasileiros e do Distrito Federal, filtrando os códigos C69.0 a C69.9 como causa básica de óbito, de acordo com a Classificação Internacional de Doenças e Problemas Relacionados à Saúde - 10ᵃ Revisão. Resultados: Houve 1.859 óbitos por neoplasia maligna de olho e anexos (C69), no Brasil, no período de 2010 a 2019, acometendo 1.062 (57,1%) homens. O sítio da neoplasia não foi especificado (C69.9) em 719 casos, representando a etiologia mais frequente no grupo C69 (38,67%). A neoplasia maligna da órbita (C69.6) foi a segunda causa mais comum de óbito (22,59%), seguida pela neoplasia maligna da retina (C69.2) (14,73%). Conclusão: O número de óbitos por neoplasia maligna de olho e anexos aumentou discretamente ao longo dos anos de 2010 a 2019, no Brasil.

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Congenital hypertrophy of the retinal pigment epithelium is a rare benign tumor of the ocular fundus that may vary according to three types. It is frequently asymptomatic and diagnosed during routine ophthalmology exam. This lesion has an important differential diagnosis with retinal disease and may be related to systemic diseases, therefore, it is essential to recognize it and keep the follow-up. In this paper, three different cases of each type of CHRPE were described and documented. Abbreviations: CHRPE = Congenital hypertrophy of the Retinal Pigment Epithelium, RPE = Retinal Pigment Epithelium, CDVA = Corrected Distance Visual Acuity, PIO = Intraocular Pressure, FA = Fluorescein Angiography, FAP = Familial Adenomatous Polyposis, RPEH-FAP = Retinal Pigment Epithelial Hamartomas Associated with Familial Adenomatous Polyposis, OCT = Ocular Coherence Tomography.

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O retinoblastoma é conhecido como o mais comum dos tumores intraoculares na infância. Além dos métodos convencionais de diagnóstico, podemos acrescentar a tomografia de coerência óptica (OCT) como um instrumento relevante na avaliação seletiva do comprometimento retiniano, dos tumores pequenos e médios do polo posterior. Os autores apresentam dois casos de retinoblastoma nos quais a OCT pode demonstrar as características morfológicas do tumor ao diagnóstico e as modificações estruturais do tecido tumoral e da retina adjacente, que ocorreram, em cada caso, após distintas modalidades de tratamento.

The retinoblastoma is known as the most common intraocular malignancy of the childhood. In addition to other diagnostic methods the optical coherence Tomography (OCT) may be considered an important tool in the evaluation of selective involvement of the retina by small and medium sized tumors at the posterior pole of the eye. The authors present two cases of retinoblastoma in which the OCT was able to demonstrate the morphological features of the tumor at diagnosis and the structural changes in tumor tissue and in adjacent retina that occurred, in each case, after different modalities of treatment.

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A 4 year-old girl with bilateral, non-familial retinoblastoma (RB) was referred to our care after primary enucleation OS and active tumor OD refractory to multiple therapies (intravenous chemotherapy, laser/cryotherapy, and I-125 plaque radiotherapy). Vitreous seeding OD, initially controlled by several sessions of Ophthalmic Artery Infusion Chemotherapy (OAIC) and periocular chemotherapy, recurred shortly thereafter. The patient underwent intravitreal (IVit) Melphalan injections achieving tumor control despite the concurrent development of keratopathy, pupillary synechiae, cataract, and necrosis of the inferior fornix and the adjacent orbital fat, all secondary to the treatments administered. Repeated amniotic membrane implants and tarsorrhaphy were performed to alleviate the symptoms. Despite being tumor free for 6 months, a poor fundus view and treatment-related complications prompted us to consider enucleation, but parents declined. Following recent negative magnetic resonance imaging (MRI), her cataract was removed. She was then found to have tumor recurrence. Her eye was enucleated 12 months ago and she recovered well from the surgery. As ocular oncology embarks in eye-preserving treatments for retinoblastoma, it is important to address the cumulative effects and associated impact of such treatments and the possibility of failure.

Uma menina de 4 anos com retinoblastoma (RB) bilateral, não-familiar foi encaminhada após enucleação OE e tumor ativo OD refratário a múltiplas terapias (quimioterapia endovenosa, laser/crioterapia e braquiterapia com I-125). Semeadura vitrea OD, inicialmente controlada por inúmeras sessões de Quimioterapia Intra-Arterial Oftálmica (QIAO) e quimioterapia periocular, recorreu em seguida. Paciente recebeu injeções intravítreas de Melphalan obtendo controle tumoral apesar do desenvolvimento concomitante de ceratopatia, sinéquias pupilares, catarata, necrose do fórnice inferior e gordura periorbitária adjacente, todos secundários aos tratamentos usados. Implantes repetidos de membrana amniótica e tarsorrafias foram realizadas para melhora sintomatológica. Apesar de estar livre de tumor por 6 meses, a baixa visibilidade do fundo e complicações terapêuticas nos levaram a considerar enucleação que foi descartada pelos pais. Após recente ressonância magnética nuclear (RMN) negativa, a catarata foi removida. Foi então detectada recorrência tumoral. O olho foi enucleado há 12 meses e ela se recuperou bem da cirurgia. Enquanto a oncologia ocular embarca em tratamentos para preservar em retinoblastoma, é importante considerar os efeitos cumulativos e impacto associado desses tratamentos, e a possibilidade de fracasso.

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Retinal vasoproliferative tumor is a rare disease that has capillary hemangioma as the most frequent diferential diagnosis. The tumor is considered to be of reactive nature. It can be idiophatic or secondary to other ocular diseases such as: uveitis, retinitis pigmentosa, sickle cell disease, previous surgery and retinopathy of prematurity. Lesions with no exsudation or visual decrease can be observed. Lesions that need treatment can be managed by on or more modalities such as cryotherapy, a variety of lasers, surgical excision, radiation, and antiangiogenic intravitreal injections.

O tumor vasoproliferativo da retina é uma lesão rara, cujo principal diagnóstico diferencial é o hemangioma capilar da retina. O tumor tem natureza reacional. Pode ser idiopático ou secundário a outras doenças como: uveítes, retinose pigmentar, retinopatia da anemia falciforme, cirurgia prévia e retinopatia da prematuridade. Lesões sem exsudação ou baixa visual podem ser observadas. Quando há indicação de tratamento este pode ser feito pela crioterapia, vários tipos de lasers, excisão cirúrgica, radioterapia e injeções intravítrea de antiangiogênicos, isoladamente ou em associação.

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PURPOSE: To study the results of cataract surgery in children with radiation-induced cataract after treatment for retinoblastoma. METHODS: Retrospective interventional case series. Six consecutive patients diagnosed with secondary cataracts due to radiation therapy for retinoblastoma. Intervention: Phacoemulsification and foldable acrylic intraocular lens implantation. Outcomes measu- red: Visual acuity, binocular indirect ophthalmoscopy and slit-lamp biomicroscopy. Aspirated lens material and aqueous humor samples were collected during surgery. RESULTS: Six uniocular children between 3 to 5 years of age at time of surgery were studied. The mean time interval between radiotherapy and cataract diagnosis was 22.3 months. The mean follow-up after surgery was 17.2 months (range: 12 to 23 months). All eyes achieved a clear visual axis after surgery allowing monitoring of the tumor status. None developed recurrence or retinoblastoma dissemination. Histopathological analysis of the aspired material showed no tumoral cells in all samples. All patients improved vision after cataract surgery. CONCLUSIONS: Phacoemulsification with acrylic intraocular lens implantation seems to be a safe, feasible, and effective method for the removal of radiation-induced cataracts in patients with treated retinoblastoma.

OBJETIVOS: Estudar os resultados da cirurgia da catarata induzida pela radioterapia para o tratamento do retinoblastoma em crianças. MÉTODOS: Estudo retrospectivo intervencional em série de casos onde seis pacientes consecutivos apresentaram catarata secundária à terapia por radiação para o retinoblastoma. Intervenção: Facoemulsificação e implante de lente intraocular acrílica dobrável. Foram avaliadas: acuidade visual, oftalmoscopia binocular indireta e biomicroscopia. Material para análise histológica do cristalino e do humor aquoso foi coletado durante as cirurgias. RESULTADOS: Seis crianças, entre 3 e 5 anos de idade, com catarata secundária à radiação para tratamento de retinoblastoma foram submetidas à cirurgia de facoemulsificação com implante de lente intraocular. A média do intervalo de tempo decorrido entre a radioterapia e o diagnóstico da catarata foi 22,3 meses. O período médio de seguimento após a cirurgia foi de 17,2 meses (intervalo: 12 a 23 meses). Todos os olhos melhoraram a visão e mantiveram eixo visual livre permitindo a fundoscopia para monitorar o tumor. Nenhum paciente evoluiu com recorrência ou disseminação do retinoblastoma. A análise histopatológica do material colhido resultou em ausência de células tumorais nas amostras. CONCLUSÕES: A cirurgia de facoemulsificação com implante de lente intraocular acrílica dobrável mostrou ser um procedimento seguro e efetivo para o tratamento da catarata induzida pela radioterapia em pacientes portadores de retinoblastoma.

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O Hemangioma racemoso da retina é uma malformação congênita caracterizada por comunicações arteriovenovas (AV) retinianas. Em cerca de 30 por cento dos casos pode haver manifestações a nível do sistema nervoso central (SNC), denominando-se então por Síndrome de Wyburn-Mason. Os autores apresentam um caso clínico de um doente do sexo masculino de 39 anos que recorre ao serviço de Urgência devido a diminuição da acuidade visual do olho esquerdo (OE) com cerca de seis dias de evolução.Apresenta melhor acuidade visual corrigida olho direito (OD): 4/10 e olho esquerdo (OE): 2/10.À fundoscopia apresenta disco óptico com limites indefinidos, alguma palidez, dilatação e tortuosidade, AV marcada bilateralmente. O hemangioma racemoso da retina é um diagnóstico clínico raro, efectuado por meio do exame oftalmológico. É importante verificar o envolvimento do SNC por meio da realização de angiorressonância magnética nuclear. Atualmente não existe nenhum tratamento preconizado, sendo importante a realização de observações periódicas para averiguação de eventuais complicações.

Racemose Haemangioma (RH) is a congenital anomaly, characterized by retinal arteriovenous(AV) malformation. About 30 percent of patients have signs of central nervous system (CNS) involvement, which has been called the Wyburn-Mason syndrome. The authors present a 39-year-old male patient, who was admitted at the emergency room with decreased visual acuity of his left eye, for the last 6 days. Ophthalmic examination was performed and showed a visual acuity of 4/10 in his right eye and of 2/10 in the left eye. Fundoscopy examination revealed pallor of both optic discs, with blurred limits and a characteristic dilation and AV connections. RH of retina is a rare entity which can be diagnosed by ophthalmic examination. Once this diagnosis is established, involvement of CNS has to be rulled out performing an angioMRI. Currently, no specific treatment has been defined, except a periodic follow-up to prevent complications.

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Relatamos cinco casos de retinocitoma diagnósticados em um período de 75 meses. A média de idade ao diagnóstico foi de 193,2 meses. Dois casos apresentavam tumor unilateral, um bilateral e dois exibiam retinoblastoma no olho contralateral. As características clínicas e oftalmoscópicas, as implicações genéticas e a necessidade de tratamento e acompanhamento contínuo dos pacientes com retinocitoma são discutidas.

Five retinocytoma cases diagnosed over 75 months are reported. The mean age at diagnosis was 193.2 months. Two cases were unilateral, one was bilateral and two exhibited retinoblastoma in the fellow eye. Clinical and ophthalmoscopic aspects, genetical implications and the need of treatment and continuous follow-up of retinocytoma patients are discussed.

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OBJETIVO: Descrever um caso de tumor vasoproliferativo associado à tuberculose ocular tratado com crioterapia e injeção de triancinolona intravítrea. DESENHO DO ESTUDO/PACIENTES E MÉTODOS: Relato de caso intervencional. Paciente do sexo feminino, 42 anos, referia inflamação do olho direito havia 1 ano, não diagnosticada, tratada com prednisona oral por 30 dias. Relatava baixa visão com o olho direito (OD) desde a infância. Negava antecedentes pessoais e familiares. Ao exame ocular, apresentava acuidade visual menor que 20/400 com o OD. Não apresentava alterações à biomicroscopia e a pressão intra-ocular era normal em ambos os olhos. A fundoscopia do OD mostrava lesão vascularizada, elevada, associada ao descolamento seroso e exsudatos duros, localizada na periferia inferior da retina. A lesão era cercada por extensa área de pontos de hiperplasia do epitélio pigmentar. A mácula apresentava diminuição do reflexo foveal. No exame de ultra-som ocular, a lesão apresentava altura igual a 2,25mm e consistência sugestiva de lesão vascularizada. Foram solicitados exames sorológicos, hemograma, RX tórax e PPD. Os exames foram normais, com exceção do PPD, considerado forte reator. A paciente foi encaminhada ao infectologista, que diagnosticou tuberculose após exame de pesquisa de BK no escarro. Em face do quadro clínico, foi feito o diagnóstico de tumor vasoproliferativo da retina associado à tuberculose ocular presumida. Iniciou-se o tratamento com esquema tríplice (rifampicina, isoniazida e piridoxina). Optou-se por tratar o tumor com crioterapia e injeção intravítrea de triancinolona (4 mg/ml). Após 30 dias, a paciente apresentava diminuição do descolamento seroso e áreas atróficas na lesão tumoral. O aspecto angiofluoresceinográfico mostrava algumas áreas de enchimento precoce da trama vascular com discreto extravasamento tardio do contraste, sem áreas de oclusão capilar. A maior parte da lesão apresentava hipofluorescência por bloqueio (proliferação...

PURPOSE: To describe a case of vasoproliferative tumor associated with presumed ocular tuberculosis treated with cryotherapy and intravitreal triamcinolone injection. STUDY DESIGN/PATIENT AND METHODS: Interventional case report. A 42-year-old female patient reported inflammation of the right eye 1 year ago, treated with oral prednisone for 30 days. She referred blurred vision in the right eye since childhood. Ophthalmologic examination showed 20/400 visual acuity in the right eye. Biomicroscopy and intraocular pressure were normal. Fundus examination showed vascularized elevated lesion, associated with serous retinal detachment and hard exudates at the inferior periphery. The lesion was surrounded by extensive area of hyperplastic retinal pigment epithelium. The macula showed attenuation of the foveal reflex. Ocular ultrasound showed a 2.25 mm height vascularized lesion. Serologic examination, hemogram and thorax RX were normal. PPD was considered strong reactor and tuberculosis was diagnosed after positive BK research. Considering the clinical aspects, the ocular diagnosis was vasoproliferative tumor associated with presumed ocular tuberculosis. Treatment with rifampicin, isoniazide and pyridoxine was started. We decided to treat the ocular tumor with cryotherapy and intravitreal triamcinolone injection (4 mg/ml). After 30 days, serous detachment was smaller and the tumor showed atrophic areas. Fluorescein angiography showed areas of vascular hyperfluorescence with slight extravasation and areas of blocked fluorescence due to RPE hyperplasia. Secondary vasoproliferative tumors are retinal glial proliferations and are associated with many ocular conditions that affect retina and choroid. Because of the great number of associated complications, with important visual loss, vasoproliferative tumors should be treated at the moment of diagnosis. Treatment of choice is cryotherapy. Intravitreal triamcinolone can be used as adjuvant treatment...

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PURPOSE: Ki-67 is a nuclear protein that is expressed at all phases of the cell cycle except the resting phase. This study is a clinicopathologic observational case report that aims to report on the cell proliferation rates, as measured by the Ki-67 antigen, in two enucleated retinoblastoma eyes. METHODS: One unilateral familial (mother with unilateral disease - patient 1) and one unilateral sporadic retinoblastoma (patient 2) patients were submitted to enucleation without previous treatment. The tumor cell proliferation rate was assessed by the Ki-67 antigen labeling index (stained cells / 100 cells) in five different fields of the tumor. RESULTS: Patient 1 was 23 months old and the tumor was exophytic with associated neovascularization of the iris; patient 2 was 6 years old and the tumor was endophytic with coarse vitreous seeds. Both enucleated eyes presented optic nerve with free surgical margins. Positive Ki-67 cell index in patient 1 varied from 75 to 90 (MD ± SD: 79.5 ± 6.61) and in patient 2 from 38 to 60 (MD ± SD: 46.6 ± 8.2). CONCLUSIONS: The familial retinoblastoma, besides the earlier age presentation, showed 45.8 percent more Ki-67 positive cells than the same stage sporadic one. This proliferation rate may explain the earlier presentation age of the tumor in the inherited disease.

OBJETIVO: O Ki-67 é antígeno nuclear que se expressa em todas as fases do ciclo celular, exceto no período de repouso. Este é um estudo de casos com correlação clínico-patológica que visa avaliar a taxa de proliferação celular, medida pelo antígeno Ki-67, em 2 olhos enucleados com retinoblastoma. MÉTODOS: Um paciente com retinoblastoma unilateral familiar (mãe com doença unilateral - paciente 1) e outro com retinoblastoma unilateral esporádico (paciente 2) foram submetidos à enucleação ocular sem outro tratamento prévio. A taxa de proliferação celular foi avaliada segundo índice obtido pela contagem de células marcadas com Ki-67, em 5 campos sob microscópia óptica (células marcadas/100 células). RESULTADOS: O paciente 1, com 23 meses de idade, apresentou tumor exofítico com neovascularização de íris associada; o paciente 2, de 6 anos, apresentou tumor de crescimento endofítico, com sementes vítreas importantes. Ambos os olhos enucleados apresentaram margens cirúrgicas do nervo óptico livres de neoplasia. O índice de células positivas no paciente 1 variou de 75 a 90 (Média ± DP: 79,5 ± 6,61), e no paciente 2, de 38 a 60 (Média ± DP: 46,6 ± 8,2). O retinoblastoma familiar, além de sua manifestação em idade mais precoce, apresentou 45,8 por cento mais células positivas que o retinoblastoma esporádico com o mesmo estadiamento. CONCLUSÃO: O retinoblastoma familiar, além de surgimento mais precoce, apresentou 45,8 por cento mais células em proliferação que o retinoblastoma esporádico em mesmo estádio. Essa taxa de proliferação pode explicar a menor idade de aparecimento do tumor nos casos de doença herdada.

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