Declining incidence and improving survival of ocular and orbital lymphomas in the US between 1995 and 2018.

This epidemiological study examined ocular and orbital lymphomas in the United States from 1995 to 2018, using data from the North American Association of Central Cancer Registries database of 87,543 patients with ocular and adnexal malignancies. We identified 17,878 patients (20.4%) with ocular and orbital lymphomas, with an age-standardized incidence rate (ASIR) of 2.6 persons per million (ppm). The incidence was the highest in the orbit (ASIR = 1.24), followed by the conjunctiva (ASIR = 0.57). Non-Hodgkin B-cell lymphoma was the most prevalent subtype (85.4%), particularly marginal-zone lymphoma (45.7%). Racial disparities were noted, with Asia-Pacific Islanders showing the highest incidence (orbit, 1.3 ppm). The incidence increased significantly from 1995 to 2003 (Average Percent Change, APC = 2.1%) but declined thereafter until 2018 (APC = - 0.7%). 5-year relative survival (RS) rates varied, with the highest rate for conjunctival lymphoma (100%) and the lowest for intraocular lymphoma (70.6%). Survival rates have generally improved, with an annual increase in the 5-year RS of 0.45%. This study highlights the changing epidemiological landscape, pointing to initial increases and subsequent decreases in incidence until 2003, with survival improvements likely due to advancements in treatment. These findings underscore the need for further research to investigate the root causes of these shifts and the declining incidence of ocular lymphoma.

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.

Clinical spectrum and clinicopathological correlation of pediatric orbital tumors: 20 years' experience from a tertiary eye care center.

PURPOSE: To study the epidemiological profile of various pediatric orbital tumors and determine their clinicopathological correlation over 20 years. METHODS: A retrospective analysis of all pediatric patients with orbital tumors from the medical records between 1995 and 2015 was done. Tumors were categorized based on their cellular origin. Demographic details, clinical and histopathological diagnoses, frequency, and clinicopathological correlation were studied. RESULTS: A total of 265 pediatric patients with orbital tumors were analyzed, of which 145 (54.7%) patients were males and 120 (45.3%) were females. The mean age was 7 years (median: 6 years; range: 10 days-16 years). The distribution of lesions in each group was as follows: vasculogenic, 76 (28.7%); lipogenic and myxoid, 66 (25%); myogenic, 31 (11.7%); optic nerve and meningeal, 22 (8.3%); lymphoid and lymphoproliferative, 18 (6.8%); secondary orbital tumors, 14 (5.2%); histiocytic, 9 (3.4%); miscellaneous, 9 (3.4%), lacrimal gland, 8 (3%); mesenchymal with neural differentiation, 6 (2%); osseous, 4 (1.5%), and fibrocystic, 2 (0.8%). The most common benign and malignant pediatric orbital tumors were dermoid cysts (65, 24.5%) and rhabdomyosarcoma (31, 11.7%). A statistically significant ( P < 0.0001) clinicopathological correlation was observed in 208 (83.2%) out of 250 biopsy-proven cases. CONCLUSION: Pediatric orbital neoplasms include a broad spectrum of benign and malignant lesions. Vasculogenic tumors constitute the majority of them, followed by lipogenic and myxoid tumors. A thorough knowledge of the incidence, clinical profile, imaging features, and histopathology of specific orbital tumors aids in accurate diagnosis and their successful management.

[OCULO-ORBITAL TUMOURS AT THE NATIONAL UNIVERSITY HOSPITAL CENTRE OF BANGUI (CNHUB), CENTRAL AFRICAN REPUBLIC, IN 2022]. / TUMEURS OCULO-ORBITAIRES AU CENTRE NATIONAL HOSPITALIER UNIVERSITAIRE DE BANGUI (CNHUB), RÉPUBLIQUE CENTRAFRICAINE, EN 2022.

Introduction: Oculo-orbital tumors are common. Their clinical and histological features are multiple. The management of oculo-orbital tumors is a real challenge in sub-Saharan Africa, especially in our context. The aim of this study was to contribute to the improvement of the management of oculo-orbital tumors at the Bangui Teaching Hospital (CNHUB). Methodology: This was a prospective, observational study of oculo-orbital tumors over 12 months from January 1 to December 31, 2022 in the ophthalmology department of the CNHUB. All consenting patients with histologically proven tumors of the eyeball and/or its adnexa were included in this study, regardless of age and sex.The parameters studied were:epidemiological: age, sex, occupation, origin;clinical: time of consultation, functional signs and associated signs, physical examination signs, risk factors;paraclinical: report of the anatomopathological examination of the operating room;therapeutic: treatment modalities (chemotherapy, surgery, palliative treatment, radiotherapy).These data were collected on a pre-established survey form for direct administration. The processing and the production of the results were carried out with Excel and Epi Info 7.0 software. Results: We included 97 patients with a mean age of 37.5 years, ranging from 2 to 70 years. There was a male predominance (sex-ratio = 1.8). Almost all patients lived in Bangui (96%). Patients under 40 years of age were most commonly affected (55%). The majority of tumors were benign (55%). Ocular pain (24%), lacrimation (22%) and orbital swelling (16%) were the main symptoms. Risk factors were mainly age (23%), diabetes (22%), HIV infection (16%) and alcohol and tobacco use (37%). The most common treatments were surgical removal (68%) and enucleation (16%). Chemotherapy was combined with surgery in 37% of cases. Conclusion: Oculo-orbital tumors are frequent in our context. Their morpho-histological presentations are varied. A multidisciplinary approach is important for a good management.

Differences in clinical features and prognosis between orbit adenoid cystic carcinoma and adenocarcinoma: a study from the SEER 18 database.

PURPOSE: To analyze the differences in clinical features, treatment methods, prognosis, and etiological factors between lacrimal gland, adenoid cystic carcinoma (LGACC), and orbit adenocarcinoma. MATERIAL AND METHODS: All cases of LGACC and orbit adenocarcinoma between 1975 and 2016 were obtained from the US National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database. RESULTS: The 3-, 5-, and 10-year overall survival (OS) rates for 118 LGACC cases were 82.2%, 70.2%, and 51.7%, respectively. The 3-, 5-, and 10-year OS rates for 29 orbit adenocarcinoma cases were 76.3%, 51.2%, and 51.2%, respectively. There was no statistically significant difference in the overall survival rate between LGACC and adenocarcinoma (P=0.566). Univariate analysis showed that the proportion of deaths was higher with increased age and year of diagnosis in LGACC (P=0.010 and P=0.000), while the death rate was higher for poorly differentiated adenocarcinoma (P=0.020). The survival rate for LGACC was lower in patients ⩾60 years of age than in those <60 years of age (P=0.035). There was no significant difference in survival rate between adenocarcinoma patients ⩾60 years and <60 years old (P=0.102). There was no significant correlation between the degree of tumor differentiation and the survival rate for LGACC or adenocarcinoma (P=0.401 or P=0.098, respectively). CONCLUSION: There is no significant difference in prognosis between LGACC and adenocarcinoma. The degree of tumor differentiation is not associated with prognosis for either LGACC or adenocarcinoma.

Orbital Tumors and Tumor like Lesions: A Hospital Based Study.

BACKGROUND: Orbital tumors have rare incidence, still they play a significant role in terms of morbidity and mortality. Orbital tumors may be primary, secondary or metastatic. These consist of benign and malignant lesions with extreme variations in pediatric and adult groups. These lesions can have acute or chronic onset, slow to rapid progression with or without bony destructions leading to vision loss, deformity and sometimes death. METHODS: This retrospective cross-sectional study was carried out in the Department of Ophthalmic Pathology and Laboratory Medicine in Biratnagar Eye Hospital. Fifty-one patients who underwent histopathological evaluation for their orbital lesions from June 2018 to December 2019 were included in the study. RESULTS: Orbital tumor and tumor like lesions comprised 27 cases (52.94%) in adults and 24 (47.06%) in paediatrics. Histopathologically, the most common pediatric benign tumor was dermoid cyst and malignant was secondary to orbital extension of retinoblastoma. Also, the most common adult benign orbital tumor was cavernous hemangioma and malignant was non Hodgkins lymphoma. The clinico-pathological accuracy for diagnosis was 68.63%. Association between age groups and nature of orbital lesions and between clinical and histopathological diagnosis was found to be statistically significant (p<0.05). CONCLUSIONS: Orbital tumors and tumor like lesions are uncommonly encountered. These masses showed significant variation in incidence in children versus adults. Combined efforts by different specialties help in early and prompt management of the orbital tumors.

Ten-year epidemiological study of ocular and orbital tumors in Chiba University Hospital.

BACKGROUND: The purpose of this study is to determine the epidemiology of tumors of the ocular adnexa and orbit in Japan. METHODS: We conducted a retrospective study on the histopathological reports in the medical records of the Chiba University Hospital from April 2009 to March 2019. Three hundred and seventy two records were examined. In addition, we examined the annual changes in the major types of tumors including malignant lymphomas and IgG4-related diseases (IgG4-RDs). RESULTS: There were 270 conjunctival or eyelid tumors with 166 benign and 104 malignant. There were 102 orbital tumors with 55 benign, 47 malignant tumors, and 21 cases of IgG4-RDs. Ten cases of adenoma (2.7%), another benign tumor, was also diagnosed. The major malignant tumors were malignant lymphoma in 74 cases, sebaceous gland carcinoma (SGC) in 28 cases, basal cell carcinoma in 15 cases, and squamous cell carcinoma in 8 cases. The SGCs were the most common malignant eyelid tumor at 54%. Among the malignant lymphomas, extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type, MALT lymphomas, was the most common at 51 cases and the second most common was the diffuse large B-cell lymphoma at 11 cases. The ratio of MALT lymphomas to that of all malignant lymphomas increased significantly with years. The serum IgG4 values were measured more often in the last 5 years (70%) than in the former 5 years (33%). CONCLUSIONS: We conclude that malignant lymphoma is a major malignant tumor in Japan and pathological biopsies should be done proactively to prevent missing IgG4-positive MALT lymphomas.

Clinico-epidemiological analysis of 1000 cases of orbital tumors.

PURPOSE: To clarify the incidence, demography and clinical features of orbital tumors diagnosed in a single institute in Japan. STUDY DESIGN: Retrospective, observational case series. METHODS: Patients with primary orbital tumors including tumor-like lesions diagnosed clinically or histopathologically at Tokyo Medical University Hospital between 1995 and 2019 were analyzed. Incidence of all orbital tumors, demographic profile and clinical features of major benign and malignant tumors were reviewed retrospectively. RESULTS: Totally 1000 cases of primary orbital tumor were diagnosed clinically or histopathologically during the study period. Benign tumors accounted for 72% and malignant tumors 28%. 55% of benign tumors and 99% of malignant tumors were proven histopathologically. The most common benign orbital tumor was idiopathic orbital inflammation (27%), followed by IgG4-related ophthalmic disease (17%), cavernous venous malformation (13%) and pleomorphic adenoma (9%). The most common malignant tumor was lymphoma (70%), followed by adenoid cystic carcinoma (7%) and solitary fibrous tumor (5%). CONCLUSIONS: Epidemiology of orbital tumors has changed by the improvement of imaging techniques, establishment of novel clinical and histopathological criteria, and changes in population age structure associated with the aging society. Currently, lymphoproliferative diseases including lymphoma and IgG4-related ophthalmic diseases form the major orbital tumors in Japan.

Incidence and trends of ophthalmic cancer in Singapore: Data from Singapore Cancer Registry.

INTRODUCTION: Limited data are available on the incidence of primary ophthalmic cancers worldwide. We describe the incidence and trends of primary ophthalmic cancers in Singapore. METHODS: Data on ophthalmic cancers diagnosed in Singapore from 1996 to 2016 were retrieved from the Singapore Cancer Registry for analysis. All were histologically proven primary ophthalmic cancers. Calculations of incidence and age-specific frequency of ophthalmic malignancy were made. RESULTS: A total of 297 cases were included, with males constituting 59.9%. The race distribution was 78.5% Chinese, 16.5% Malay, 3.7% Indians and 1.3% others. There was an overall increase in ophthalmic malignancies. The mean age of onset was 47.4 years. The most common cancers were retinoblastoma (93.3%) in patients younger than 15 years, and lymphoma (71.3%) in patients aged 15 years and older. There has been an increase in lymphomas from 16.7% in 1968-1995 to 71.3% in 1996-2016 in those aged 15 years and older. The most common types of ophthalmic cancer according to location are lymphoma of the orbit, conjunctiva, cornea and lacrimal gland; retinoblastoma of the retina; and malignant melanoma of the choroid and ciliary body. CONCLUSION: Our study reported the incidence and trends of ophthalmic cancer in the Singapore population and showed an overall increase in ophthalmic malignancies in Singapore from 1996-2016. A substantial increase in lymphomas over the last 2 decades was noted. The data could aid clinicians, epidemiologists and policymakers in implementing strategies to address trends in ophthalmic cancers and spur aetiological research to improve quality of life in patients with such cancers.

Ocular involvement in metastatic and systemic malignancies is not rare.

BACKGROUND: Metastatic disease to the eye most commonly involves choroid followed by orbit leading to varied ocular manifestations. By comparison, it is relatively rarer than primary malignancies of eye as well as metastasis in other parts of the body. AIM: The aim of this study is to evaluate the common eye and orbital structures involved in secondary ocular and metastatic disease, to describe its clinical manifestations and outline the management done. METHODS: A retrospective study of newly diagnosed cases of ocular metastasis in last 2 years conducted in our recently established department of ocular oncology at a tertiary eye care hospital in Nepal. Demography, age and sex distribution were noted. The patients were segregated into those with secondary or metastatic ocular malignancies. Detail study on the metastatic disease to eye was made in regards to presenting symptoms, signs, primary site of cancer, and the treatment done. Details of the investigations done, like biopsy and imaging were also recorded. RESULTS: There were a total of 28 patients, whose age group ranged from 9 years to 69 years with median age of 43 years. Females constituted 46% of total patients. Both the eyes were involved in 9 patients (32%). Eye was secondarily involved by paranasal sinus tumors and Non Hodgkin lymphoma (7 patients each). Ocular metastasis was commonly seen from broncogenic carcinoma in four and breast carcinoma in three patients. Simultaneous metastasis to other parts of the body was also seen in 61% of our patients. Diminution of vision in 49% was the most common presenting feature followed by proptosis in 16% and palpable mass in 14% of patients. Orbit in 43% cases is the commonest ocular structure involved. Histopathologic diagnosis was done in 32% only while rest was based on imaging alone. The most common treatment done was chemotherapy in 57% patients. CONCLUSION: Ocular metastasis can display a wide variety of clinical and imaging features and therefore a high degree of suspicion is required. It is often associated with simultaneous metastasis to other parts of the body as well, hence the importance of earlier diagnosis and metastatic workup.

Orbital and ocular adnexal lymphoma: a review of epidemiology and prognostic factors in Taiwan.

PURPOSE: To investigate the clinical features, prognostic outcomes of patients with orbital and ocular adnexal lymphoma (OALs) in Taiwanese cohort. METHODS: Total 112 patients with OALs were retrospectively reviewed. Demographic information such as age, gender, lymphoma subtype, tumor location and treatment modalities were recorded. The primary endpoints were disease-specific survival (DSS), and progression-free survival (PFS). RESULTS: The mean patient age was 59.0 ± 15.5 years (range, 23-92 years). The major histopathologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 76 patients (67.9%), followed by diffuse large B-cell lymphoma (DLBCL) (9.8%), follicular cell lymphoma (FL) (8.0%), and small lymphocytic lymphoma (SLL) (5.4%). The anatomical locations for OALs were the orbit (44 patients, 39.3%), the conjunctiva (31 patients, 27.7%), the lacrimal gland (28 patients, 25.0%), and the eyelid (8 patients, 7.1%). With a mean follow-up time of 74.5 ± 59.8 months (range 6-342 months), the DSS for all patients were 93.1%, 87.7%, and 68.8% at 60, 120, and 180 months' follow-up, respectively. The PFS at 60, 120, and 180 months' follow-up were 86.2%, 61.2%, and 44.6%, respectively. Regarding the analysis of prognostic factors, patients with high grade lymphoma subtype and advanced stage exhibited a worse prognosis. CONCLUSIONS: MALT type lymphoma constitutes most of OALs in Taiwan and occurs more frequently than in Western countries. Patients with MALT lymphoma, FL, SLL and earlier stage have favorable outcomes compared with patients of high grade lymphoma and Ann Arbor stage IV lymphoma.

Ocular adnexal lymphoma in Denmark: a nationwide study of 387 cases from 1980 to 2017.

BACKGROUND: Nationwide studies of ocular adnexal lymphoma (OAL) are very rare in the literature, and knowledge on incidence, subtype distribution and long-term survival data is limited. This is the largest national study of OAL to date. This study sought to find information on incidence, changes in incidence, clinical findings, distribution of subtypes, survival and prognostic factors. METHODS: Patients diagnosed with OAL from January 1, 1980 to December 31, 2017 were identified in Danish registers, and clinical as well as survival data were collected. The data were analysed with Kaplan-Meier plots and log-rank test. RESULTS: 387 patients were included in the study. The major lymphoma subtypes were extranodal marginal-zone B cell lymphoma (EMZL) (55%), diffuse large B cell lymphoma (DLBCL) (13%), mantle cell lymphoma (MCL) (11%) and follicular lymphoma (FL) (10%). OAL is a disease of the elderly (median age 69 years). The incidence of lymphoma of the ocular adnexal region has increased significantly throughout the time period of the study (Pearson correlation coefficient, r=0.65; P<0.001). In the period 1980-1984, the incidence was 0.086 per 100 000, which increased to 0.307 per 100 000 in the period 2013-2017. Low-grade, low-stage primary lymphomas were treated with radiotherapy, whereas patients with high-stage, high-grade and/or relapsed disease were treated with chemotherapy. Low-grade subtypes EMZL (89%) and FL (56%) had better 10-year disease-specific survival than the high-grade lymphomas DLBCL (38%) and MCL (31%)(p<0.001). CONCLUSION: OAL is increasing in incidence in the Danish population for unknown reasons. However, the prognosis for most OAL is favourable, as highlighted in this national long-term study.

Nationwide trends in the incidence of orbital lymphoma from 1999 to 2016 in South Korea.

BACKGROUND/AIMS: Lymphomas are the most frequent neoplasm of the orbit. However, the epidemiology of orbital lymphomas is not well reported. This study aimed to provide a population-based report on the epidemiology of orbital lymphomas and measure the trends in the incidence of orbital lymphoma cancer in South Korea. METHODS: Nationwide cancer incidence data from 1999 to 2016 were obtained from the Korea Central Cancer Registry. Age-standardised incidence rates and annual percent changes were calculated according to sex and histological types. The analysis according to the Surveillance, Epidemiology, and End Results summary stage classifications was performed from 2006 to 2016. Survival rates were estimated for cases diagnosed from 1999 to 2016. RESULTS: A total of 630 patients (median age: 54 years) with orbital lymphoma in the orbital soft tissue were included in this study. The age-standardised incidence rates increased from 0.03 to 0.08 per 100 000 individuals between 1999 and 2016, with an annual percent change of 6.61%. The most common histopathological type of orbital lymphoma was extra marginal zone B cell lymphoma, accounting for 82.2% of all orbital lymphomas during 1999-2016, followed by diffuse large B cell lymphoma (9.2%). Five-year, 10-year and 15-year overall survival (OS) of orbital lymphoma was 90.8%, 83.8% and 75.8%, respectively. OS showed a significant decrease as age increased and no significant differences between men and women. CONCLUSION: The incidence rate of orbital lymphoma is very low in South Korea. However, the incidence rate has increased over the past years. Orbital lymphomas have a worse prognosis as age increases.

Histopathological Features and Ann Arbor Stage in Periocular Lymphoma.

BACKGROUND: To evaluate biodata, symptoms/signs, lymphoma type, localization, stage level, treatment choice and outcome of ocular adnexal lymphoma (OAL). PATIENTS AND METHODS: A single-center retrospective analysis of 56 patients with OAL was performed from 1998 to 2018. RESULTS: OAL involved the orbit in 44.6%, the conjunctiva in 32.1%, the lacrimal apparatus in 14.3% and the eyelid in 8.93%. Extranodal marginal zone B-cell lymphoma (EMZL) was found in 60.7%, follicular lymphoma (FL) in 21.4%, diffuse large B-cell lymphoma in 7.14%, mantle cell lymphoma in 5.36% and chronic lymphatic leukaemia in 5.36% patients. No relapse was seen in 76%. EMZL and FL had a significantly better overall survival compared to other lymphoma types (p=0.002). Patients with Ann Arbor stage IE had a significantly better prognosis than those with stages higher than IE (p=0.048). CONCLUSION: Our data suggest that clinicopathological features such as Ann Arbor stage influence survival.

Histopathologic spectrum of paediatric eye and ocular adnexal tumours: A 10-year review from a referral centre in Nigeria.

BACKGROUND: Tumours of the eye and adnexa demonstrate great histologic variety and constitute a serious threat to vision especially in children. AIMS: The study aims to review the epidemiologic and pathologic characteristics of tumours of the eye and ocular adnexa in the paediatric age group (0-14 years). METHODS: All the cases entered into the departmental records as tumours of the eye and ocular adnexa over a 10-year period in the age group 0-14 years were extracted. The patients' request cards with all relevant Haematoxylin & Eosin (H & E)-stained histology slides were retrieved. All the slides were reviewed and the cases were classified in accordance with the 4th edition of the WHO Classification of Tumours of the Eye (2018). The collected data were subjected to descriptive statistical tabulation and analysis. RESULTS: A total of 104 tumours of the eye and ocular adnexae were diagnosed in the paediatric age group, accounting for 40.5% of all eye and ocular adnexal tumours diagnosed over the study period. The male to female ratio was 1.7:1 and malignant tumours greatly outnumbered benign tumours by a ratio of 5.5:1. Majority (76%) of the tumours occurred in the retina with retinoblastoma representing all the tumours diagnosed in this location. Rhabdomyosarcoma was the most common paediatric orbital tumour accounting for over half (53.8%) of all tumours in the orbit. Tumours of the conjunctiva and the eyelid were infrequent with benign soft tissue tumours (vascular, neural and lipomatous tumours) being the major tumours at these sites. CONCLUSION: Retinoblastoma is the single most common tumour in this age group.

Evaluation of cytology and histopathology for the diagnosis of feline orbital neoplasia: 81 cases (2004-2019) and review of the literature.

OBJECTIVE: To provide an updated overview of feline orbital neoplasia, to compare diagnostic utility of cytology and histopathology, and to evaluate minimally invasive sampling modalities. PROCEDURES: A medical records search was performed to identify cats with orbital neoplasia. Data were collected regarding signalment, diagnosis, vision status, imaging modalities, and sample collection methods. A reference population with orbital neoplasia was also identified via literature search for comparison with regard to final diagnosis. RESULTS: Eighty-one cats met selection criteria and 140 cases were identified in the literature. In the study and reference populations, respectively, diagnoses were grouped as follows: round cell tumors 47% and 24%, epithelial tumors 38% and 40%, mesenchymal tumors 14% and 34%, and neurologic origin tumors 1% and 2%. The most common diagnoses in both groups were lymphoma and squamous cell carcinoma (SCC). Feline restrictive orbital myofibroblastic sarcoma (FROMS) was common in the reference population but not diagnosed in the study population. Cytology results were available for 41 cats; histopathology results were available for 65 cats. Both cytology and histopathology results were available for 25 cats, in 44% of which cytologic results were overturned. No significant complications were associated with any sampling method. Lack of cats with multiple samples available for histopathology limited comparison between tissue sampling methods. CONCLUSIONS: Orbital neoplasia is common in cats, with round cell and epithelial tumors diagnosed most commonly in the study population. Histopathology is superior to cytology in providing a definitive diagnosis. Minimally invasive tissue biopsy techniques appear to be safe and effective.

Orbital metastases from breast cancer: A single institution case series.

BACKGROUND AND OBJECTIVES: Among orbital tumors, metastatic lesions have a prevalence of 1% to 13%; on the other hand, breast cancer is the most common malignancy causing orbital metastases. The aim of this study is to present our experience dealing with orbital metastases caused by breast cancer, to assess characteristics and clinic-pathological data of patients suffering from this rare occurrence and to find indexes related with their prognosis and survival. METHODS: Records of 28 patients diagnosed with orbital metastases from breast cancer at the Department of Ophthalmology, Humanitas Clinical and Research Centre of Milano over a 27-year period (1992-2018) were retrieved and analyzed. RESULTS: Mean patients' age at breast cancer diagnosis was 56.29 ± 14.63 years. Mean time interval between breast cancer diagnosis and orbital metastasis occurrence was 5 ± 4.17 years. All lesions were estrogen receptor-positive; 79% of patients harbored progesterone receptor-positive lesions. Interestingly, the majority of deceased patients presented orbital lesions with MIB-1 index >50% (P = .0265) and had concomitant lung metastases (P = .0452). CONCLUSIONS: The occurrence of orbital metastasis from breast cancer represents a challenging finding. Patients' clinical picture can include exophthalmos, edema, tumefaction, proptosis and/or diplopia. Significant symptomatic improvement can be achieved through surgery and other adjuvant treatments, such as radiation therapy and chemotherapy.

Epidemiology, outcomes, and prognostic factors of orbital lymphoma in the United States.

PURPOSE: We performed an epidemiological study of orbital lymphoma in the United States to determine how histological subtypes confer differing prognosis, and understand other factors associated with survival. METHODS: All patients in the Surveillance, Epidemiology and End Results database diagnosed with a histologically confirmed orbital lymphoma between 1973 and 2014 were included. Exclusion criteria included diagnosis at autopsy and the presence of other malignancies. Measures included patient demographic information, histological subtype and treatment modalities. Outcomes included overall and disease specific survival. RESULTS: Of the 1504 cases identified, 702 were male (46.7%, mean age: 64.4 years, standard deviation [SD]: 15.3) and 802 were female (53.3%, mean age: 67.5 years, SD: 14.3). Mucosal associated lymphoid tissue (MALT) (49.5%) and diffuse large B cell lymphoma (DLBCL) (19.5%) were the two most common histologic subtypes. MALT lymphoma conferred the best prognosis (10-year cancer specific survival [CSS] 90.2%, 95% Confidence Interval [CI] 87.4% - 93.1%) and DLBCL conferred the worst prognosis (10-year CSS 68.6%, 95% CI 62.5% - 75.3%) (p < .001, log-rank test). Older age (Hazard Ratio [HR]: 3.71, 95% Confidence Interval [CI]: 2.94-4.66, p < .001), male sex (HR: 1.22, 95% CI: 1.039-1.441, p = .015), no radiation (HR: 1.72, 95% CI: 1.46-2.02, p < .001) and DLBCL histology were significant predictors of worse overall survival. CONCLUSIONS: DLBCL histology confers the worst outcomes whereas MALT lymphoma confers the best outcome in orbital lymphoma. Age, gender, and radiation treatment also influence survival. These epidemiological results can be used clinically to communicate outcomes on the basis of patient characteristics and disease histology.

[Does orbital exenteration still has a place in 2019?] / L'exentération orbitaire a-t-elle encore sa place en 2019 ?

INTRODUCTION: Orbital exenteration is a radical anatomically and psychologically disfiguring procedure. It is mostly performed for management of orbital cancers or cancers with orbital involvement. The lack of benefit in terms of overall survival and the development of new molecular therapies (targeted therapies, immunotherapy) in recent years leads us to question its use. The goal of our review is to answer to the following question: is orbital exenteration a viable procedure in 2019? MATERIALS AND METHODS: A literature review was performed using the PUBMED and MEDLINE databases. The following terms were used then crossed with each other: "orbital exenteration", "exenterated socket", "overall survival", "life expectancy", "orbital reconstruction", "socket reconstruction". Oncology articles from the past 15 years were included and separated into those in the oculoplastic literature and those in the ENT literature. RESULTS: Nineteen articles were included in this review. Eyelid tumours represent the main etiology of orbital exenteration. Basal cell carcinoma is the most frequently incriminated tumor, while sebaceous carcinoma and conjunctival squamous cell carcinoma are the most frequently encountered in Asian series. Non-conservative orbital exenteration is the most prevalent surgery performed. Orbital reconstruction depends on the surgeon's speciality: healing by secondary intention and split thickness skin grafts are mostly performed by oculoplastic surgeons, whereas regional or free flaps are mostly performed by ENT surgeons. Cerebrospinal fluid leakage is the most common intraoperative complication, encountered in 0 to 13 % of cases. The most common postoperative complications are ethmoid fistula and infection of the operative site, encountered in 0 to 50 % and 0 to 43 % of cases respectively. Orbital exenteration allows surgical resection of R0 tumors in 42.5 % to 97 % of cases. Overall survival following orbital exenteration is 83 % (50.5-97) and 65 % (37-92) at 1 and 5 years respectively. Identified risk factors for poor overall survival are: age, tumor histology (worse prognosis with choroidal melanoma, better prognosis with basal cell carcinoma), non-R0 surgical resection, locally advanced tumors (size>20mm, BCVA<20/400 and the presence of metastases at diagnosis). Recent studies have demonstrated favorable outcomes when managing locally advanced basal cell carcinoma, lacrimal gland cancer and conjunctival melanoma with targeted therapies or immunotherapies without performing orbital exenteration. CONCLUSION: Orbital exenteration remains a major part of our therapeutic arsenal. Although orbital exenteration has failed to demonstrate any overall survival benefit, it allows satisfactory local control of the disease with an increasingly less invasive procedure. The development of targeted therapies and immunotherapies may change our therapeutic decisions in the future.