Orbital mucosa-associated lymphoid tissue (MALT) lymphoma as the initial presentation in patients with hepatitis C virus infection

ABSTRACT Hepatitis C virus infection may be implicated in 12.7% of ocular adnexal marginal zone lymphomas. We present the first case of an orbital-systemic mucosa-associated lymphoid tissue lymphoma that responded to hepatitis C virus medical treatment. A 62-year-old male with a right-sided orbital mass was diagnosed with stage IIA orbital marginal zone lymphoma in addition to hepatitis C virus infection based on clinical, imaging, laboratory, and histological examinations. The systemic and orbital responses were achieved 1 year after undergoing hepatitis C virus treatment with glecaprevir/pibrentasvir. The association between the hepatitis C virus infection and orbital-systemic mucosa-associated lymphoid tissue lymphoma is relevant. Accordingly, patients with orbital mucosa-associated lymphoid tissue lymphoma should be assessed for hepatitis C virus seroreactivity for therapeutic and prognostic purposes.

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Mucormicosis orbitorrinocerebral: el manejo más allá de la cirugía. Informe de caso clínico y revisión de la literatura / Rhino-orbital-cerebral mucormycosis: management beyond surgery. Clinical case report and literature review

Introducción: se presenta el caso clínico de un paciente con mucormicosis orbitorrinocerebral y revisión de la literatura. Caso clínico: se trata de hombre de 45 años que consultó por síntomas nasosinusales manejado ambulatoriamente con múltiples antibióticos sin mejoría, por lo cual se realizó manejo quirúrgico con toma de muestras que revelaron hifas compatibles con mucormicosis. Cuando acudió a la institución presentaba extensión de la infección a todas las cavidades nasales y parte de la base del cráneo. En la búsqueda de la inmunosupresión, se encontró una diabetes de novo de difícil control. Discusión: la mucormicosis orbitorrinocerebral es una enfermedad altamente invasiva que requiere un manejo multidisciplinario, cirugías seriadas y extendidas, antimicóticos tópicos y sistémicos. El pronóstico mejora y la evolución puede ser favorable cuando se logra controlar la causa de la inmunosupresión, en este caso la diabetes. Conclusiones: es requisito fundamental realizar un tratamiento multidisciplinario en el abordaje de estos pacientes, tanto en la especialidad clínica como en la quirúrgica, psicosocial, nutricional y de rehabilitación.

Introduction: It is the clinical case of a patient with rhino-orbital-cerebral mucormycosis and the review of the literature. Clinical case: We present the case of a previously healthy young man who started with sinonasal symptoms and hyphae compatible with mucormycosis were found in the initial samples of outpatient care. When he came to the institution, the infection had spread to all the nasal cavities and part of the skull base. Discussion: Searching for baseline immunosuppression, only difficult-to-control diabetes and de novo diagnosis were found. It required multidisciplinary management, serial and extended surgeries, topical, venous and oral antifungals. The prognosis improved and the evolution became favorable when glycemic control was achieved. Conclusion: It is a fundamental requirement to carry out a multidisciplinary work for the approach to these patients, both in the clinical, surgical, psychosocial, nutritional and rehabilitation specialties.

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.

Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

Bilateral metastases to the extraocular muscles from small cell lung carcinoma / Metástases bilaterais aos músculos extraoculares de um carcinoma de pequenas células do pulmão

ABSTRACT Bilateral orbital metastases restricted to the extraocular muscles (EOMs) are exceedingly rare. We report a case of bilateral extraocular muscle metastases from a small cell lung carcinoma and provide a review of the relevant literature. A 56-year-old smoker presented with proptosis, motility changes, and a relative afferent pupillary defect of the left eye, with a previous history of a small cell lung carcinoma. An orbital computerized tomography scan revealed a mass restricted to the left medial rectus. An incisional biopsy confirmed metastasis. Visual acuity of the left eye decreased rapidly, and right globe proptosis became evident. Orbital magnetic resonance imaging at two months follow-up showed marked left orbital mass enlargement and a new right lateral rectus mass. The patient was maintained on palliative care and died from metastatic disease-related complications.

RESUMO As metástases orbitárias bilaterais restritas aos mús­culos extraoculares são extremamente raras. Os autores apresentam um caso de metástases bilaterais, localizadas aos musculares extraoculares com base num carcinoma de pequenas células do pulmão e revisão da literatura relevante. Um homem, fumador, de 56 anos recorreu ao serviço de urgência por proptose, alterações de motilidade ocular extrínseca e um defeito pupilar aferente relativo do olho esquerdo, com história pessoal de carcinoma de pequenas células do pulmão. A tomografia computadorizada orbitária revelou uma massa restrita ao reto medial esquerdo. Uma biópsia incisional confirmou o diagnóstico de metástase. A acuidade visual do olho esquerdo diminuiu rapidamente e surgiu uma proptose do globo ocular direito. A ressonância magnética orbitária aos dois meses de seguimento revelou um aumento da massa orbitária esquerda e uma nova massa no reto lateral direito. O paciente foi mantido em cuidados paliativos e faleceu devido a complicações relacionadas com doença metastática.

Giant frontal lipoma mimicking frontal mucocele / Lipoma frontal gigante mimetizando mucocele frontal

ABSTRACT Lipoma is a usually painless benign adipose tumor. Nevertheless, it can cause compression of adjacent structures, depending on its location. Mucoceles are benign cystic lesions in the frontal-ethmoidal region, caused by chronic obstruction of the paranasal sinus and consequent inflammatory processes. The increase of mucocele diameter caused by inflammation often results in bony degradation and reabsorption, increasing pressure on adjacent structures, including the orbit and the base of the skull, leading to possibly serious consequences. Here we report a case of lipoma mimicking a frontal mucocele, managed surgically at the Ophthalmological Hospital of Anapolis.

RESUMO O lipoma é um tumor do tecido adiposo de caráter benigno, usualmente indolor, porém que pode gerar compressão de estruturas adjacentes, dependendo da sua localização. A mucocele consiste em uma lesão cística de caráter benigno, na região fronto-etmoidal, devido à obstrução crônica dos óstios dos seios paranasais e consequente processo inflamatório. O aumento do diâmetro da mucocele por inflamação muitas vezes causa degradação e reabsorção ósseas, o que pode elevar a pressão em estruturas adjacentes como órbita e base de crânio, causando intercorrências possivelmente graves. Neste estudo relatamos um caso de lipoma simulando mucocele frontal, com propedêutica cirúrgica.

Angioleiomyoma in the Orbital Apex: A Case Report

A 56-year woman presented eyeball pain and blurred vision. MRI revealed a small well-delineated solid tumor in the apex of right orbit with optic nerve compression. Intraoperatively, the tumor was found very fibrous, hypervascular and adhesive to surrounding structures. The tumor was completely removed with the combination of endoscopic and microscopic technique. Patient experienced transient oculomotor nerve palsy, which completely recovered 3 months after surgery. Herein we report a rare case of angioleiomyoma in the orbital apex.

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

Clinical features and prognosis of lacrimal gland adenoid cystic carcinoma with high-grade transformation / 中华实验眼科杂志

Objective To summarize the clinical characteristics and prognosis of lacrimal gland adenoid cystic carcinoma with high-grade transformation (LACC-HGT).Methods A retrospective study was adopted.Seventeen patients with lacrimal gland adenoid cystic carcinoma (LACC) were collected from August 2008 to March 2017 in Tianjin Medical University Eye Hospital.Hematoxylin-eosin staining and immunohistochemical staining were performed on tumor sections of 17 LACC patients.According to the pathology results,the patients were divided into LACC-HGT group (6 patients) and non-LACC-HGT (NLACC-HGT)group (11 patients),and the clinical characteristics and prognosis of the two groups were analyzed by using Fisher's exact test and Log-Rank test.Results The medical histories,clinical features,imaging features,TNM staging and treatment protocols were not significantly different between the two groups (all at P＞ 0.05).The 2-year local recurrence rate,5-year distant metastasis rate and 5-year death rate of patients with LACC-HGT were significantly higher than patients with NLACC-HGT (all at P＜0.05).Survival analysis results showed that the survival time of LACC-HGT group was obviously shorter than that of NLACC-HGT group.Conclusions LACC-HGT accelerate the invasion process of local recurrence and distant metastasis,and enhance the death rate.The detection of HGT components should be considered in the process of LACC diagnosis.

Extraocular Muscles Involvement as the Initial Presentation in Metastatic Breast Cancer / 한국유방암학회지

Orbital metastasis is a rare event, and metastatic disease affecting the extraocular muscles is an even less frequent complication of solid tumors. Herein, we report an unusual case of ptosis as the initial presentation of an invasive breast cancer. A 68-year-old woman presented with III and VI partial nerve paresis, secondary to a compressive retrobulbar mass. Magnetic resonance imaging revealed an infiltrative lesion involving the extraocular muscles. Tissue biopsy yielded a result compatible with metastasis to the orbit, with immunohistochemistry analysis suggesting breast as the primary organ. Mammography identified an area of architectural distortion; stereotactic wire-guided biopsy confirmed the result of the previous orbital biopsy. A positron emission tomography scan demonstrated disseminated disease. Palliative chemotherapy with bone-modulating agents and subsequent hormonal therapy was proposed. Unfortunately, the patient did not respond to therapy and died 38 months after diagnosis.

Tumor primario quístico de la órbita: A propósito de un caso de hidatidosis infantil / Primary orbital cystic tumor: A case of hydatidosis in a child

RESUMEN Los tumores quísticos primarios de la órbita, causados por infestaciones parasitarias como el Equinococcus granulosus son raros. Al ser el Perú zona endémica para este parásito, presentamos el caso de una niña de 10 años de edad, proveniente de una zona rural de Huancavelica, con proptosis axial irreductible del globo ocular derecho, de curso progresivo, con pérdida de la agudeza visual e imágenes tomográficas de la órbita en relación a masa tumoral de apariencia quística. Los exámenes serológicos fueron negativos, la paciente fue sometida a resección quirúrgica con resultado histopatológico de membranas anhistas correspondientes a quiste hidatídico. Al año de seguimiento no se evidencia recurrencia local ni sistémica. En conclusión, ante una tumoración orbitaria quística en pacientes pediátricos, se debe de considerar como diagnóstico diferencial la hidatidosis orbitaria, sobre todo si proceden de zonas endémicas.

ABSTRACT Primary orbital cystic tumors caused by Echinococcus granulosus are rare. As this parasite is endemic in Peru, we report the case of a 10-year-old girl from a rural area of Huancavelica, who presented with progressive and irreducible axial proptosis of the right eye and loss of vision. Orbital tomographic images demonstrated a tumor mass with a cystic appearance. Serologic tests were negative. The patient underwent surgical resection, and histopathologic analysis revealed anhistic membranes, corresponding to hydatid cysts. During one year of follow-up, no evidence of local or systemic recurrence was observed. In conclusion, orbital hydatidosis should be considered in the differential diagnosis of a cystic orbital tumor in pediatric patients, especially if the patient is from an endemic area.

Metástasis intraorbitárias de cáncer de mama / Breast cancer orbital metastasis

Objetivo: Describir un caso raro de una paciente previamente diagnosticada con cáncer de mama que evolucionó después de algunos años con metástasis intraorbitaria. Métodos: Relato del caso y revisión de literatura. Resultados: La correlación del diagnóstico previo de neoplasia de mama y la presencia de múltiples lesiones en la órbita y regiones adjacentes ha permitido concluir que la paciente presentaba enfermedad metastásica. Las metástasis se presentan como manifestaciones cada vez más frecuentes en enfermos oncológicos. La órbita y estruturas próximas constituyen topografías poco usuales de diseminaciónde células neoplásicas y manifestaciones iniciales pueden mimetizar otras condiciones, retrasando el diagnóstico. Como se trataban de pequeñas lesiones, se ha optado por radioterapia como tratamiento único. Después del término la paciente evolucionó con regresión de sintomatología. Conclusiones: Aunque infrecuentes, manifestaciones oculares pueden ocurrir en enfermos oncológicos y la investigación de enfermedad metastático de la órbita es esencial para el seguimiento adecuado.

Objective: Description a rare case of a patient with previous diagnosis of breast cancer that evolved years after with orbital metastasis. Method: Case report and literature review. Results: The relation between the previous diagnosis of breast cancer and the presence of multiple orbital and adjacent lesions allowed concluding that the patient presented metastatic disease. Metastasis presented as a common manifestation in oncological patients. Orbit and neighboring structures constitute unusualsite of neoplastic cells dissemination and initial manifestations could mimic other conditions, which can slow diagnosis. As they were small lesions that are close to noble structures, it was opted by performing exclusive radiotherapy. After the end of treatment, the patient referred improvement of her symptoms. Conclusions: Although unusual, ocular manifestations could occur in oncological patients and orbit metastatic disease investigation is essential to the proper follow.

Oncocytoma of the lacrimal gland: a case report / Oncocitoma de glândula lacrimal: relato de caso

ABSTRACT Here we describe a rare case of a benign tumor in the lacrimal gland of a healthy 4-year-old girl. Mild proptosis was the only abnormality observed on clinical examination. Magnetic resonance imaging of the right orbit revealed an oval, solid, well-circumscribed, homogeneous mass extending from the lacrimal gland and measuring 2.5 × 2.3 × 1.7 cm without any evidence of invasion into adjacent bones. The lesion was surgically excised and histological analyses defined the diagnosis of oncocytoma of the lacrimal gland. Although rare, oncocytoma should be included in the differential diagnosis of lacrimal gland tumors.

Resumo Nós descrevemos um raro caso de tumor benigno na glândula lacrimal em uma criança sadia de 4 anos de idade. Clinicamente, a paciente apresentava apenas uma discreta proptose. A ressonância nuclear magnética (RNM) de órbita direita revelou a presença de uma massa oval, sólida, bem-circunscrita, homogênea, se extendendo a partir da glândula lacrimal, medindo 2,5 cm x 2,3 cm x 1,7 cm, sem nenhum sinal evidente de invasão a estrutura óssea adjacente. A lesão foi cirurgicamente removida e analizada histopatologicamente, sendo estabelecido o diagnóstico de oncocitoma de glândula lacrimal. Apesar de raro, o oncocitoma deve ser incluído no diagnóstico diferencial de qualquer tumor originado da glândula lacrimal.

Clinical practice of transnasal endoscopic operation for retrobulbar lesions / 中华耳鼻咽喉头颈外科杂志

Objective@#To summarize the skill and experience of transnasal endoscopic operation for retrobulbar lesions.@*Methods@#Seven patients aged from 25 to 67 years old diagnosed as retrobulbar lesions who underwent transnasal endoscopic operation in Department of Otorhinolaryngology Head and Neck Surgery, Xiangya Hospital between January 2013 and October 2016 were retrospectively analyzed. Two males and five females were included in this study. Five patients underwent transnasal endoscopic operation via media rectus-inferior rectus space, with the other 2 cases via media rectus-superior rectus space.@*Results@#Total lesion removal was achieved in 6 of 7 patients, while 1 patient underwent subtotal removal of the lesion. The visual acuity and visual field improved in 3 cases. The pathological examination showed hemangioma(5 cases), bone cyst(1 case) and fibroma(1 case). All patients were followed up for 9 months to 4 years without complications such as eye movement disorder or blindness, except for 1 case with preoperatively proptosis occurred postoperatively transient diplopia. There was no recurrence in 6 patients with total lesion removal, and the patient underwent subtotal removal of fibroma did not undertake operation again.@*Conclusion@#Transnasal endoscopic operation for retrobulbar lesions is a minimally invasive, safe and effective operatiiv method, which could be taken via different surgical approaches according to the size and location of the lesion.

Unilateral Ptosis with Bilateral Incomplete Ophthalmoplegia as the Initial Presentation in Metastatic Cancer / 이화의대지

Orbital metastases are rare and predominantly unilateral occurrences. Bilateral metastases affecting the extraocular muscles are extremely rare. A few case reports of bilateral metastases to extraocular muscles described binocular diplopia with conspicuous bilateral external ophthalmoplegia as an initial symptom. We report a case in which unilateral ptosis was an initial symptom and bilateral incomplete ophthalmoplegia was found on initial neurologic examination in invasive ductal carcinoma of the breast. The patient had hormone receptor-positive breast cancer, and so was treated by hormonal therapies and closely monitored. The presence of a secondary orbital lesion presents many difficulties of differential diagnosis and treatment. A thorough neurologic examination to detect ocular manifestations is most important for localization and broad differential diagnosis including mechanical orbital metastatic lesion.

Exenteração orbitária: série de casos / Orbital Exenteration: a series of cases

RESUMO Objetivo: Descrever os casos de exenteração orbitária de um hospital terciário brasileiro. Métodos: Estudo retrospectivo, envolvendo pacientes submetidos à exenteração orbitária no Hospital das Clínicas da Faculdade de Medicina de Botucatu, entre os anos de 1993 a 2016. As cirurgias foram realizadas sob anestesia geral, por equipe multidisciplinar composta por oftalmologistas, otorrinolaringologistas e cirurgiões de cabeça e pescoço. Resultados: Foram estudados 14 casos de exenteração orbitária, com média de idade de 63,36 ± 13,18 anos e nove homens (64,3%). Todas cirurgias foram realizadas para tratamento de tumores malignos, sendo mais frequente o carcinoma espinocelular (7 casos - 50,0%). Os sítios primários mais frequentes foram as pálpebras (50,0%), seguida pela conjuntiva (28,6%). A maioria das cirurgias foram do tipo exenteração estendida (57,1%), com cicatrização por granulação espontânea (64,3%). A sobrevida em 1 ano foi de 78,6% e em 5 anos de 71,4%. Conclusão: O carcinoma espinocelular foi a principal causa de indicação de exenteração orbitaria, sendo as pálpebras o sítio primário mais frequente. O procedimento mais realizado foi a exenteração estendida, com a grande maioria alcançando margens livres.

ABSTRACT Objective: To describe causes of orbital exenteration in a Brazilian tertiary hospital. Methods: A retrospective study was done, involving patients submitted to orbital exenteration at the Clinical Hospital of Botucatu Medical School, between the years of 1993 to 2016. The surgeries have been performed under general anesthesia, by a multidisciplinary team, composed by ophthalmologists, otolaryngologists and head and neck surgeons. Results: Fourteen cases of orbital exenteration occurred in the period of the study, with a mean age of 63.36 ± 13.18 years and nine were men (64.3%). All exenteration were due to malignant tumors, being more frequent the squamous cell carcinoma (7 cases - 50.0%). The most common primary sites were the eyelids (50.0%) followed by the conjunctiva (28.6%). The majority of the surgeries was extended exenteration type (57.1%) and most of the reconstructions was made by spontaneous granulation (64.3%). The survivor rate in 1 year was 78,6% and in 5 years was 71.4%. Conclusion: The main cause of orbital exenteration was squamous cell carcinoma and the most frequent primary site was the eyelids. Extended exenteration was necessary for the majority of cases, most of them with free margins.

Apresentação incomum e agressiva de melanoma intraorbitário / Unusual and aggressive presentation of intraorbital melanoma

RESUMO O objetivo dos autores é relatar um caso de melanoma intraorbitário de apresentação atípica e agressiva, formando grande massa dolorosa de aspecto eritematoso e inflamatório projetando-se da órbita esquerda com o globo ocular danificado em seu ápice. A análise da peça identificou melanoma maligno com componentes celulares epitelióide, fusocelular e anaplásico.

ABSTRACT The purpose of this study is to report a intraorbital melanoma case with atypical and aggressive presentation, forming a large painful mass with erythematosus and inflammatory aspect protruding from the left orbit with eyeball damaged at its peak. Piece analysis identified malignant melanoma compound of epithelioid, spindle and anaplastic cells.

Extraconal cystic schwannoma mimicking an orbital dermoid cyst / Schwannoma cístico extraconal simulando um cisto dermóide orbitário

ABSTRACT To our knowledge, we report the first case of an extraconal orbital cystic schwannoma in Brazilian literature. The tumor grew slowly and progressively and was associated with minimal eccentric proptosis and diplopia. The radiologic study (orbital computed tomography) revealed a rounded, encapsulated, and extraconal cystic mass in the superior medial region of the right orbit in the supraorbital and supratrochlear nerve topography. An anterior orbitotomy with full excision of the tumor was performed, and the histopathology examination revealed that the tumor was a schwannoma. After the surgery, the patient experienced total remission of his symptoms.

RESUMO Os autores relatam o primeiro caso de schwannoma orbitário extraconal cístico relatado na literatura brasileira. O tumor apresentou aparecimento lento e progressivo, associado a proptose excêntrica e diplopia leves. O exame de imagem (tomografia computadorizada de órbita) revelou uma lesão nodular, arredondada, capsulada, extracônica, cística, na região súpero-medial de órbita direita, na topografia do nervo supraorbitário e supra-troclear. O paciente foi submetido à orbitotomia por via anterior, com exérese integral da tumoração. O exame histopatológico revelou o diagnóstico de schwannoma. Após a cirurgia, o paciente teve regressão total dos sintomas.

Linfoma no Hodgkin con infiltración ocular: a propósito de un caso / Non-Hodgkin’s lymphomawith ocular infiltration: a case report

Fundamento: el linfoma no Hodgkin es un cáncer del tejido linfoide incluido en el sistema inmunológico, comprende una variedad de enfermedades con características histológicas, inmunológicas, genéticas y clínicas diferentes. Objetivo: describir la evolución de un enfermo con linfoma no Hodgkin con manifestaciones oftalmológicas. Caso clínico: paciente de 71 años de edad, que acudió a consulta de Hematología por primera vez, que manifestaba aumento de volumen de la región lateral izquierda del cuello; de 3-4 cm de diámetro, consistencia firme, no doloroso a la palpación, que después de realizarle estudios clínicos, analíticos e histopatológicos se concluyó como un linfoma no Hodgkin con infiltración conjuntival. Se describe y presenta el caso por lo infrecuente de esta localización. Conclusiones: el linfoma no Hodgkin, puede manifestarse de forma multicéntrica con localizaciones en todos los órganos como se pudo observar en el enfermo.

Background: non-Hodgkin’s lymphoma is a lymphoid tissue cancer in the immune system. It includes a variety of diseases with different histological, immunologic, genetic and clinical characteristics. Objective: to describe the progress of a sick patient with Non-Hodgkin´s lymphoma, who also has ophthalmological manifestations. Clinical case: a male 71-year-old patient who went to Hematology consultation for the first time. He presented a volume rise in the left-hand lateral region of the neck, 3-4 cms in diameter, strong consistency, no painful when palpating. After carrying out clinical, analytic, and histopathologic studies, it was defined as a Non-Hodgkin’s lymphoma.with conjunctival infiltration. The case is described and presented due to the infrequency of that localization. Conclusions: non-Hodgkin’s lymphoma can become apparent in a multicentric way, with localizations in all organs, as it could be seen in this patient.