Analysis of the mortality by eye cancer from 2010 to 2019 in Brazil / Análise da mortalidade no Brasil por câncer ocular de 2010 a 2019

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.

A case of metastatic non-teratoid ocular medulloepithelioma in an adult horse

This case reports an eight-years-old horse that presented a unilateral tumor in the left ocular orbital region diagnosed on the basis of clinical signs, gross anatomopathological and histopathological investigations. The affected area was exenteration and submitted to histopathological evaluation and was diagnosed as a non-teratoid medulloepithelioma. This is a rare ocular neoplasm that usually affects young individuals – and was composed by neuroepithelium arranged in tubules and Flexner-Wintersteiner rosettes without presence of heteroplastic components. The animals died and was submitted to post-mortem evaluation, revealing presence of medulloepithelioma metastases at the internal region of the head, lymph nodes, lungs, and liver. This neoplasm usually presents a benign frame; however, the case herein described shows that it can be highly malignant and aggressive since it can cause extensive lesions and spread metastases to visceral organs.

A case of metastatic non-teratoid ocular medulloepithelioma in an adult horse

This case reports an eight-years-old horse that presented a unilateral tumor in the left ocular orbital region diagnosed on the basis of clinical signs, gross anatomopathological and histopathological investigations. The affected area was exenteration and submitted to histopathological evaluation and was diagnosed as a non-teratoid medulloepithelioma. This is a rare ocular neoplasm that usually affects young individuals and was composed by neuroepithelium arranged in tubules and Flexner-Wintersteiner rosettes without presence of heteroplastic components. The animals died and was submitted to post-mortem evaluation, revealing presence of medulloepithelioma metastases at the internal region of the head, lymph nodes, lungs, and liver. This neoplasm usually presents a benign frame; however, the case herein described shows that it can be highly malignant and aggressive since it can cause extensive lesions and spread metastases to visceral organs.(AU)

Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma.

Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. We are reporting 7 cases of an unusual clinical variant of HVL with primarily periorbital edema. All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. Most cases were initially misinterpreted as inflammatory processes including cellulitis, arthropod bite reactions, and periorbital lupus erythematosus. The biopsy of these lesions revealed an atypical lymphocytic infiltrate predominantly distributed in the deep dermis and in subcutaneous fat. Immunohistochemistry studies revealed a cytotoxic T-cell (CD8) profile. All cases were associated with Epstein-Barr virus infection. Our study presents a rare clinical variant of HVL with predominant periorbital edema. This variant could potentially be overlooked and misdiagnosed as an inflammatory condition; thus, it needs to be included in the differential diagnosis of periorbital edema in young patients.

Tumor indiferenciado da lâmina fusca / Undifferentiated tumor of the lamina fusca

Na reunião da Associação Pan-americana de Patologia Oftálmica, realizada em Los Angeles CA, 10 de Outubro de 1991, no DOHENY EYE INSTITUTE, C.O. Degrazia propôs o nome de LOFONEUROGONIOMA para um tumor solitário intra-ocular, indiferenciado, originado nas células da lâmina fusca, com células indiferenciadas de expressiva diferenciação para a linhagem schwannocítica, melanocítica e neuroendócrina. Em face do trimorfismo, o patologista pode ser conduzido para os diagnósticos de melanoma, schwannoma maligno ou outro tipo de tumor. O exaustivo estudo do tumor apresentado, através da microscopia eletrônica, da histoquímica e da imunohistoquímica permitiu a formulação da seguinte hipótese: uma célula indiferenciada em repouso, a lofoneurogô- nia, segue as linhagens melanocítica, schwannocítica e neuroendócrina. A diversidade de células dentro de um tumor, o continuus intratumor, responsável pela estrutura em mosaico de muitas neoplasias, além da multiclonalidade resultante de mitoses atípicas, pode ser explicada por essa hipótese. É dessa maneira que se torna compreensível a classificação de Callender para os melanomas intra-oculares nos tipos celulares fusiforme A, fusiforme B, epitelióide, fasciculado e misto, num verdadeiro continuus intertumores. Para justificar a designação proposta, e para enquadrar o caso num grupo taxonômico, foram usadas duas bases classificatórias: 1o ­ histogênica, isto é, correlacionar as células do tumor com as células normalmente existentes nas membranas oculares, no caso, a lâmina fusca; 2o ­ embriogênica isto é, delimitar um grupo de tumores cuja base, no desenvolvimento do embrião, é a crista neural (AU)

In the meeting of the Panamerican Association of Ophtalmic Pathology, held in the Doheny Eye Institute in Los Angeles, CA on Oct 10 1991, C.O. Degrazia proposed the name LOPHONEUROGONIOMA for an undifferentiated intraocular solitary tumor, originating from the cells of the lamina fusca, with undifferentiated cells of expressive differentiation for the schwannian, melanocitic and neuroendocrine lines. Because of the trimorphism, the pathologist may be led to the diagnosis of melanoma, malignant schwannoma or other type of tumor. The exhaustive investigation of the presented tumor through electronmicroscopy, histochemistry, and immunohistochemistry allowed the formulation of the following hypothesis: an undifferentiated cell at rest, the lophoneurogonia, follows the melanocitic, schwannian, and neuroendocrine lines. The diversity of cells inside a tumor responsible for the mosaic structure of many neoplasias, besides the multiclonality resulting from atypical mitoses, can be explained by this hypothesis. This also elucidates Callender's classification of intraocular melanomas in cell types fusiform A, fusiform B, epithelioid, fasciculated and mixed, in a true intertumor continuum. In order to justify the proposed designation, and to fit the case into a taxonomic group, two classificatory bases were used: first, a histogenic one, correlating tumor cells with normally existing cells; and second, embriogenic which bases is the neural crest (AU)

Patología ocular para el patólogo general / Ocular pathology for the general pathologist

La Patología Ocular incluye el estudio de los procesos o enfermedades que afectan el globo ocular (las estructuras que lo componen) y sus anexos. Esta especialidad ha avanzado en el tiempo en la misma proporción que lo han hecho la Patología General y la Oftalmología, de las cuales se nutren. El objetivo de la presente revisión es la actualización de términos y procedimientos de utilidad para los patólogos generales, que reciben muestras relacionadas con el campo de la Oftalmología. Los especialistas en Oftalmología y sus pacientes se beneficiarán de un óptimo estudio y, con el consiguiente reporte final se accederá a un tratamiento adecuado. Se revisan así mismo, los tópicos más resaltantes y comunes en la práctica diaria de la Patología Ocular.

Neoplasias de la órbita: neoplasias de tejidos blandos / Soft tissues orbital neoplasms

Las neoplasias de tejidos blandos en la órbita tienen una presentación relativamente rara, por lo que la experiencia con estas neoplasias es bastante limitada. Este artículo reporta las características de los casos revisados en el Departamento de Anatomía Patológica Ocular de la APEC atendidos de 1958 a 1998.

Tumores disontogénicos del globo ocular y sus anexos. I. Introducción, aspectos básicos y hamartomas / Disontogenic tumors of the eye and ocular anexa. Introduction, basic principles and hamartomas

Los tumores disontogénicos son lesiones proliferativas que se originan como un trastorno del desarrollo e incluyen una variedad de neoformaciones: Hamartomas, coristomas y el complejo grupo de los teratomas. La mayoría de estas lesiones son benignas con excepción de los teratomas. Son entidades algunas de ellas frecuentes en la clínica y otras son excepcionales. El tratamiento es quirúrgico y el pronóstico es bueno en la mayoría de los casos. Es un grupo de lesiones poco conocidas en la clínica oftalmológica. Se hace una revisión completa del tema en tres partes. Los hamartomas pueden ser simples o complejos y las características histopatológicas y la taxonómicas dependen de sus componentes tisulares. Afectan a la órbita, párpados, conjuntiva, córnea y túnicas internas

Tumores y pseudotumores de globo ocular / Tumors and pseudotumors of the eye

Se hace una revision retrospectiva y transversal de las biopsias remitidas en el Servicio de Anatomia Patologica en el Hospital de Clinicas Universitario de la ciudad de La Paz entre enero de 1980 a diciembre de 1990. Registrandose 8050 biopsias quirurgicas, 99 casos remitidos del Instituto Nacional de Oftalmologia que fueron clasificados de acuerdo a parametros establecidos por la OMS, identificandose 9 casos de tumores malignos, 7 retinoblastomas y 2 melanomas. Que constituyen el 7,07 // y el 2,02 // respectivamente, siendo mas frecuente en el sexo masculino y en el grupo etareo de 1-4 anos.

Situaçäo do retinoblastoma nos últimos 17 anos em Curitiba / Situation of retiblastoma on last 17 years in Curitiba

Realisou-se uma revisäo de retinoblastoma em 4 hospitais de Curitiba. Encontrou-se 92 casos nos últimos 17 anos, onde analisou-se: idade em se estabeleceu o diagnóstico, sexo, tempo decorrido entre o início dos sintomas e o seu diagnósticos, sintomas mais comuns, lateralidade e extensäo e óbito destes pacientes. Conclui-se que o tempo decorrido entre o diagnóstico e o início dos sintomas é elevado, pela falta de esclarecimento da populaçäo e falta de trabalho direcionado à classe médica. Isto deverá ser melhorado com a formaçäo de equipes multidisciplinares envolvendo oftalmologistas, pediatras, oncologistas, radioterapeutas, geneticistas, psicólogos, sanitaristas e membros da comunidade

Endophytic vs exophytic unilateral retinoblastoma: is there any real difference?

A retrospective review of 297 cases of enucleated and histologically proven unilateral retinoblastoma with a minimum of 5-year follow-up was analyzed to see if there were any clinical or prognostic differences between endophytic and exophytic type retinoblastoma. Endophytic retinoblastoma (181 cases) was found more often than exophytic (116 cases). The following features were not associated with the clinical appearance of either endophytic or exophytic tumors: sex of patient, right vs left eye, propensity for bilateral development, initial sign or symptom, presence of rubeosis, preoperative metastasis, optic nerve invasion, orbital recurrence, survival of the patient, length of follow-up, or age at diagnosis. Three features were found that correlated with type: a disproportionately higher percentage of patients with endophytic retinoblastoma had a positive family history; a disproportionately high percentage of patients with exophytic retinoblastoma developed glaucoma; and choroidal invasion occurred significantly more often in patients who had exophytic retinoblastoma than in those who had endophytic retinoblastoma.