A retrospective analysis of the management and surgical treatment of orbital lesions: Outcomes and rationale.

The orbital cavity is a subject of interest for various specialists, and achieving optimal outcomes requires comprehensive, multidisciplinary management. This study aims to report 10 years of experience in the preoperative, surgical, and postoperative care of patients with orbital lesions, examining their clinical, radiological, and anatomopathological features and outcomes. A retrospective review of 125 patients who underwent surgical treatment for intraorbital masses between January 2012 and December 2021 was performed. Outcome measures included postoperative diplopia, exophthalmos, decimal visual acuity, eyeball position, ocular motility, operative time, complications, and aesthetic results. A total of 107 patients were included. All cases were discussed with a neuroradiologist to determine the best therapeutic approach based on preoperative imaging. Preoperative diplopia was linked to extraconal (p = 0.03) and anterior (p = 0.001) lesions, and exophthalmos and visual acuity deterioration were associated with intraconal (p = 0.02; p = 0.03) and retrobulbar (p = 0.001; p = 0.02) lesions. Complications (11.2%) included diplopia, worsened visual acuity, postoperative blepharoptosis, and postoperative ectropion. Of the patients, 80.4% reported an "excellent" aesthetic outcome. This study underscores the importance of a multidisciplinary approach based on a thorough analysis of preoperative imaging. Periorbital approaches tailored to the lesion's three-dimensional location enables safe access to most intraorbital lesions, resulting in minimal complications and good aesthetic results.

Comprehensive analysis of orbital lymphoma in a Turkish cohort: clinical characteristics, histological subtypes, treatment modalities, prognostic factors, and implications for management.

The study analysed the clinical characteristics, treatment approaches, and survival outcomes of 97 consecutive patients with orbital lymphoma (OL) over a 25-year period at. The median age of the patients was 57.6 years, and 59.8% (n = 58) were male. Marginal zone lymphoma constitutes the most prevalent subtype, accounting for 67% of cases, whereas other common subtypes include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, and T-cell lymphomas. Unilateral involvement was observed in the majority of cases (72.3%). Common clinical presentations included mass (30.9%), swelling (26.8%), and epiphora (11.3%). Of the patients, 7.2% received rituximab alone, 14.4% received radiotherapy alone, 48.5% received chemotherapy, 27.8% received radiotherapy plus rituximab, 22.7% received radiotherapy plus chemotherapy, and 5.2% underwent surgery as the first-line treatment. During a median follow-up of 4.3 years, 15.5% of patients experienced relapse or disease progression. The 5-year and 10-year progression-free survival rates were 84.1% and 79.1%, respectively. This study contributes to our understanding of OLs and provides a foundation for further investigations in this field. Male gender, presence of B symptoms, advanced stage, secondary orbital lymphoma, aggressive histological subtype, and elevated serum lactate dehydrogenase levels were associated with poorer (either inferior or worse) progression-free survival.

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.

Biological identity of orbital cavernous venous malformations

ABSTRACT Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

Sebaceous carcinoma in Western Australia: A registry-based study of site-related outcomes.

Sebaceous carcinoma is a rare cutaneous malignancy which is typically regarded as relatively aggressive and has traditionally been subdivided into periorbital or extraorbital tumours. We conducted a retrospective review of all cases of sebaceous carcinoma reported to the Western Australian Cancer Registry between 1987 and May 2019. The incidence of sebaceous carcinoma in Western Australia has increased over the last three decades, with extraorbital tumours being much more common than periorbital tumours. Very few sebaceous carcinomas have led directly to the death of patients; however, adverse outcomes were more likely with periorbital tumours, in particular local recurrence and the need for major surgical intervention.

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.

A signature of structural MRI features at 3 Tesla allows an accurate characterization of orbital cavernous venous malformation.

OBJECTIVES: To differentiate OCVM from other orbital lesions using structural MRI. METHODS: This IRB-approved a historical-prospective cohort single-center analysis of a prospective cohort that included consecutive adult patients presenting with an orbital lesion undergoing a 3T MRI before surgery from December 2015 to May 2021. Two readers blinded to all data read all MRIs assessing structural MRI characteristics. A univariate analysis followed by a stepwise multivariate analysis identified structural MRI features showing the highest sensitivity and specificity when diagnosing OCVM. RESULTS: One hundred ninety-one patients with 30/191 (16%) OCVM and 161/191 (84%) other orbital lesions were included. OCVM were significantly more likely to present with a higher signal intensity than that of the cortex on T2WI: 26/29 (89.7%) versus 28/160 (17.5%), p < 0.001, or with a chemical shift artifact (CSA): 26/29 (89.7%) versus 16/155 (10.3%), p < 0.001, or to present with a single starting point of enhancement, as compared to other orbital lesions: 18/29 (62.1%) versus 4/159 (2.5%), p = 0.001. The step-wise analysis identified 2 signatures increasing performances. Signature 1 combined a higher signal intensity than that of the cortex on T2WI and a CSA. Signature 2 included these two features and the presence of a single starting point of enhancement. Sensitivity, specificity, and accuracy were 0.83, 0.94, and 0.92 for signature 1 and 0.97, 0.93, and 0.93 for signature 2, respectively. CONCLUSION: Structural MRI yields high sensitivity and specificity when diagnosing OCVM. KEY POINTS: • Structural MRI shows high sensitivity and specificity when diagnosing orbital cavernous venous malformation. • We identified two signatures combining structural MRI features which might be used easily in routine clinical practice. • The combination of higher signal intensity of the lesion as compared to the cortex on T2WI and of a chemical shift artifact yields a sensitivity and specificity of 0.83 and 0.94 for the diagnosis of orbital cavernous venous malformation, respectively.

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

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.

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.

Endoscopic approach for orbital apex lesions: case series and review of the literature.

OBJECTIVE: The transnasal endoscopic approach may provide better visualisation and a safer approach to the orbital apex. This study presents a case series of orbital apex lesions managed by this approach. METHOD: This study was an eight-year retrospective analysis of seven patients who were operated on for orbital apex lesions in two tertiary medical centres. RESULTS: Complete tumour removal was performed in three patients and partial removal was performed in four patients. Visual acuity improved in three patients, remained stable in one patient and decreased in the other two patients. The visual field improved in four patients and did not change in two patients. Complications included worse vision and visual fields in 28.6 per cent of patients and late enophthalmos (of -1.25 ± 4.6 mm) in 2 patients. CONCLUSION: The transnasal approach to orbital apex lesions in selected cases may provide a rational alternative to transorbital surgery. Complete tumour removal should be weighed against the risk of damage to the optic nerve.

Comparison between 7 Tesla and 3 Tesla MRI for characterizing orbital lesions.

PURPOSE: Characterizing orbital lesions remains challenging with imaging. The purpose of this study was to compare 3 Tesla (T) to 7 T magnetic resonance imaging (MRI) for characterizing orbital lesions. MATERIALS AND METHODS: This prospective single-center study enrolled participants presenting with orbital lesions from May to October 2019, who underwent both 7 T and 3 T MRI examinations. Two neuroradiologists, blinded to all data, read both datasets independently and randomly. They assessed general characteristics of each orbital lesion as well as image quality and presence of artifacts. Comparison between both datasets was made using Fisher exact test. RESULTS: Seven patients (4 women, 3 men) with a median age of 52 years were enrolled. Orbital lesion conspicuity was better scored at 7 T compared to 3 T MRI, with 3/7 lesions (43%) scored as very conspicuous at 7 T compared to 0/7 lesion (0%) at 3 T, although the difference was not significant (P = 0.16). Delineation of lesion margins was better scored at 7 T compared to 3 T with 3/7 lesions (43%) scored as very well delineated on 7 T compared to 0/7 lesions (0%) at 3 T, although the difference was not significant (P = 0.34). Details of internal structure were better assessed at 7 T compared to 3 T, with 4/7 lesions (57%) displaying numerous internal details compared to 0/7 lesions (0%) at 3 T (P = 0.10). Internal microvessels were visible in 3/7 lesions (43%) at 7 T compared to 0/7 lesions (0%) at 3 T (P = 0.19). CONCLUSION: Although no significant differences were found between 7 T and 3 T MRI, assumably due to a limited number of patients, our study suggests that 7 Tesla MRI might help improve the characterization of orbital lesions. However, further studies with more patients are needed.

Virtual planning and navigation for targeted excision of intraorbital space-occupying lesions: proposal of a computer-guided protocol.

The purpose of this study was to present an innovative approach for the preoperative assessment and intraoperative targeted excision of masses occupying the intraorbital space based on multimodal image fusion, segmentation, virtual models, digital planning, and navigation. Nineteen patients were studied and underwent surgery using the presented workflow, in both open and endoscopic procedures. Three main scenarios were standardized for the application of computer-guided surgery: single masses of the superior-lateral compartment, single masses of the inferior-medial compartment, and multifocal masses. An operative protocol was devised, and the accuracy of the osteotomies was analysed. All patients were managed successfully by applying the same protocol. No intraoperative complications were reported. The accuracy of the osteotomies was evaluated as a surrogate endpoint for the overall precision of surgery, showing average discrepancies of <1 mm for lateral marginotomies and <0.5 mm for endoscopic osteotomies. This study outlines an operative workflow for the implementation of virtual models to excise orbital masses, enhancing in-depth preoperative understanding of the anatomical relationships within the orbital space and increasing precision in both open and endoscopic approaches.

Ocular adnexal mucosa-associated lymphoid tissue lymphoma: a narrative review.

Lymphoma is the most common primary tumor of the orbit, accounting for 55% of all orbital malignancies. When divided into histopathological subtypes, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises the largest proportion. Clinical manifestations are unspecific, but in patients with slow-growing painless orbital mass, or red conjunctival lesion suggestive of 'salmon patch', ocular adnexa lymphoma (OAL) should be suspected. Although the pathogenetic mechanism of ocular adnexal MALT lymphoma (OAML) is not yet fully understood, the relationship between OAML and Chlamydia psittaci has been hypothesized recently, similar to that between gastric MALT lymphoma and Helicobacter pylori. This suggests a new treatment option for OAML; bacterial eradication therapy with systemic antibiotics. Several other treatment methods for OAML have been introduced, but no treatment guidelines have been established yet. In this article, we summarize the current knowledge on the clinical features, pathogenesis, diagnostic methods, therapeutic strategies, and prognosis of OAML.

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.

Clinical Assessment and Lesion-Specific Management of Orbital Vascular Malformations.

The systematic classification of vascular disease as proposed and refined by the International Society for the Study of Vascular Anomalies (ISSVA) divides vascular pathology first into tumors and malformations. Malformations are described as simple and complex, where simple malformations contain a single vascular system and complex malformations comprised of multiple vascular systems. Arteriovenous malformations are considered in terms of inflow characteristics which are primarily responsible for the key management challenges. Management utilizing endovascular embolization and/or surgical resection is often employed; however, recurrence can occur, particularly in diffuse cases. There may be an increasing role for systemic antiangiogenic therapy in such cases. Lymphaticovenous malformations are divided into the principle components on the lymphatic and venous sides for clarity of discussion. Lymphatic malformations are described morphologically as macrocystic and microcystic, and physiologically in terms of the processes responsible for growth. In both cases, surgical options are challenging and local therapeutics intended to close large luminal spaces in the case of macrocystic and to slow biological signaling for growth in microcystic. Venous malformations are described physiologically in terms of flow and distensibility, as volume plays a critical role in the limited space of the orbital cavity. Combined embolic-surgical approaches can be effective for management. More complicated, combined lesions can be managed by dividing the lesion into principal components and treating each appropriately.

Intravoxel incoherent motion (IVIM) 3 T MRI for orbital lesion characterization.

OBJECTIVES: To determine the diagnostic accuracy of MRI intravoxel incoherent motion (IVIM) when characterizing orbital lesions, which is challenging due to a wide range of locations and histologic types. METHODS: This IRB-approved prospective single-center study enrolled participants presenting with an orbital lesion undergoing a 3-T MRI prior to surgery from December 2015 to July 2019. An IVIM sequence with 15 b values ranging from 0 to 2000 s/mm2 was performed. Two neuroradiologists, blinded to clinical data, individually analyzed morphological MRIs. They drew one region of interest inside each orbital lesion, providing apparent diffusion coefficient (ADC), true diffusion coefficient (D), perfusion fraction (f), and pseudodiffusion coefficient (D*) values. T test, Mann-Whitney U test, and receiver operating characteristic curve analyses were performed to discriminate between orbital lesions and to determine the diagnostic accuracy of the IVIM parameters. RESULTS: One hundred fifty-six participants (84 women and 72 men, mean age 54.4 ± 17.5 years) with 167 orbital lesions (98/167 [59%] benign lesions including 54 orbital inflammations and 69/167 [41%] malignant lesions including 32 lymphomas) were included in the study. ADC and D were significantly lower in malignant than in benign lesions: 0.8 × 10-3 mm2/s [0.45] versus 1.04 × 10-3 mm2/s [0.33], p < 0.001, and 0.75 × 10-3 mm2/s [0.40] versus 0.98 × 10-3 mm2/s [0.42], p < 0.001, respectively. D* was significantly higher in malignant lesions than in benign ones: 12.8 × 10-3 mm2/s [20.17] versus 7.52 × 10-3 mm2/s [7.57], p = 0.005. Area under curve was of 0.73, 0.74, 0.72, and 0.81 for ADC, D, D*, and a combination of D, f, and D*, respectively. CONCLUSIONS: Our study showed that IVIM might help better characterize orbital lesions. KEY POINTS: • Intravoxel incoherent motion (IVIM) helps clinicians to assess patients with orbital lesions. • Intravoxel incoherent motion (IVIM) helps clinicians to characterize orbital lymphoma versus orbital inflammation. • Management of patients becomes more appropriate.

Orbit Solitary Fibrous Tumor: A Proposed Risk Prediction Model Based on a Case Series and Comprehensive Literature Review.

Solitary fibrous tumors (SFTs) of the orbit are rare. In order to further characterize the clinical and pathologic features of solitary fibrous tumor arising at this anatomic site, 12 cases of orbital SFTs were analyzed in conjunction with a review of 263 cases reported from the English literature in order to develop a risk prediction model. SFTs of the orbit were equally distributed between males (n = 5) and females (n = 7) with a mean patient age of 46.8 years (median 44.5 years; range 18-76 years) at initial diagnosis. The patients typically presented with swelling or mass around the orbit, with proptosis (n = 10), ptosis (n = 5), and visual changes (n = 6). Tumors were orbital (n = 10) or upper eyelid (n = 2). Mean tumor size was 2.5 cm (median 2.6 cm). Microscopically, the tumors were characterized by cytologically bland spindle cells with patternless growth, hypocellular and hypercellular areas, variable amounts of collagen, and ectatic, branching blood vessels. By immunohistochemistry, all cases had a strong nuclear STAT6 expression. All patients were initially managed with excision or biopsy, three with presurgical embolization. The two patients with biopsy only had persistent disease (mean 37.2 months), but a third patient developed distant bone metastasis at 86.9 months. Overall mean follow-up was 73.1 months: 9 patients are alive or dead without disease (mean 77.9 months), two patients with persistent disease, and one patient with metastatic disease at last follow-up (102 months). Incorporating cases sufficiently reported in the literature, a risk prediction model based on age > 45 years, tumor size > 3 cm, tumor necrosis, mitoses of > 4/2 mm2, moderate to high cellularity, and moderate to severe pleomorphism allows for risk stratification for the development of local recurrence and distant metastasis. In conclusion, orbital SFTs are rare, but can be reliably diagnosed based on the presence of characteristic morphologic features and STAT6 immunohistochemistry. Orbital tumors tend to show a higher frequency of local recurrence than distant metastasis, which can be predicted by a risk stratification model unique to orbital tumors. With late disease common, long term clinical follow-up is recommended.

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.

Orbital and Eyelid B-Cell Lymphoma: A Multicenter Retrospective Study.

BACKGROUND: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). METHODS: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. RESULTS: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8-32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6-83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 (p = 0.0112) and 9.239 (p = 0.0094), respectively. CONCLUSIONS: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

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.