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.

Clinicodemographic profile of orbital exenteration in a tertiary eye care center - A 20-year experience.

PURPOSE: This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current times and to compare these results with previous published data. METHODS: It was a retrospective study. All exenterations performed at a tertiary eye care center over a period of 20 years (from January 2001 to June 2020) were retrospectively evaluated. Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathologic diagnosis. RESULTS: A total of 352 cases (males:females = 222:130) who underwent exenteration were identified. Patients age ranged from 11 months to 87 years (mean: 43.86 years, median: 50 years). The most common indication for exenteration was found to be eyelid malignancy in 54.36%, followed by retinoblastoma in 18.75% and primary orbital tumors in 14.49%. Out of 156 cases of eyelid malignancies requiring exenteration, squamous cell carcinoma (SCC) was the most common histologic subtype ( n = 94, 60.26%), followed by sebaceous gland carcinoma ( n = 40, 25.64%) and basal cell carcinoma ( n = 20, 12.82%). The most common primary orbital tumors requiring exenteration were adenocystic carcinoma of the lacrimal gland in adults and rhabdomyosarcoma in the pediatric age group. Benign conditions requiring exenteration included fulminant fungal orbital infections and lymphangioma among others. CONCLUSION: The number of exenterations performed have significantly increased in terms of absolute numbers. However, the ratio of exenteration to other tumor-related surgeries, mainly excision biopsy, has reduced compared to that reported from a previous study. The most common indication in our study remains eyelid malignancy followed by intraocular malignancy. However, SCC has emerged as the most common tumor histologic subtype requiring exenterations.

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.

Cirugía en los tumores orbitarios: experiencia de 10 años / Surgery on orbital tumors: 10 years experience

Se presentan 35 pacientes con lesiones expansivas intraorbitarias operados en un período de 10 años en el servicio de neurocirugía del Hospital ”Hermanos Ameijeiras” mediante abordajes transcraneales convencionales y la técnica de Orbitotomía lateral de Krõnlein modificada. Se estudiaron las variables edad, sexo, síntomas de presentación, diagnostico histológico, localización topográfica, complicaciones perioperatorias y morbi- mortalidad. En el estudio etiológico e histológico se conoció que el primer lugar corresponde a la patología tumoral (65.7 por ciento) y dentro de ella el meningioma representando el 31.4 por ciento. La mayoría de las lesiones fueron benignas (82,8 por ciento). El resultado quirúrgico fue evaluado de satisfactorio en el 79 por ciento de los casos. Se demostró que la técnica modificada propuesta resulta ventajosa al permitir un acceso rápido a lesiones intracónicas, del ápex y con extensión intracraneal.

Rhabdomyosarcoma of the head and neck: a clinicopathological and immunohistochemical analysis of 29 cases

Rhabdomyosarcoma is a malignant tumor occurring more frequently in the childhood. The purpose of this study was to analyze the clinicopathological and immunohistochemical features of rhabdomyosarcomas of the head and neck (RHNs). Twenty nine patients treated in a single institution were selected. The histological slides were reviewed and the tumors were classified. The immunohistochemical reactions were performed using antibodies against vimentin, desmin, myogenin, MyoD1, AE1/AE3, p53, PCNA, Ki67, C-erbB2, FAS and CDK4. The mean age was 14.3 years. The nonparameningeal site was affected in 16 cases (55.2 percent). Eleven cases (37.9 percent) affected parameningeal sites and 2 cases the orbit. The p53 was positive in 4 cases (13.8 percent), CDK4 in 10 cases (34.5 percent), C-erbB2 in 19 cases (70.4 percent), FAS in 9 cases (31 percent), PCNA in 28 cases (96.5 percent) and Ki67 in 16 cases (55.2 percent). The overall survival was 28.7 percent in 5 and 10 years, and p53 expression may be related with poor prognosis.

Rabdomiossarcoma é um tumor maligno que ocorre mais frequentemente na infância. O objetivo deste estudo foi analisar as características clinicopatológicas e imunohistoquímicas dos rabdosiossarcomas de cabeça e pescoço. Vinte e nove pacientes tratados em uma única instituição foram selecionados. As lâminas histológicas foram revisadas e os tumores foram classificados. As reações imunohistoquímicas foram realizadas usando anticorpos contra vimentina, desmina, miogenina, MyoD1, AE1/AE3, p53, PCNA, Ki67, C-erbB2, FAS e CDK4. A idade média dos pacientes foi de 14,3 anos. Localização não-parameningeal foi o sítio mais afetado correspondendo a 16 casos (55,2 por cento). Onze casos (37,9 por cento) afetaram sítios parameningeais e em 2 casos a órbita. p53 foi positivo em 4 casos (13,8 por cento), CDK4 em 10 casos (34,5 por cento), C-erbB2 em 19 casos (70,4 por cento), FAS em 9 casos (31 por cento), PCNA em 28 casos (96,5 por cento) e Ki67 em 16 casos (55,2 por cento). A sobrevida global foi 28,7 por cento em 5 e 10 anos, e a expressão de p53 pode estar relacionado ao pior prognóstico.

Processos expansivos orbitoesfenoidais: estudo anatomopatológico de 82 casos / Orbito-sphenoidal expansive processes: an anatomopathological study of 82 cases

OBJETIVO: Descrever os processos expansivos orbitoesfenoidais diagnosticados no Laboratório de Anatomia Patológica da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre - Complexo Hospitalar Santa Casa de Porto Alegre durante o período de 15 anos, avaliando sua frequência relativa aos demais processos tumorais. MÉTODOS: Foi realizado levantamento estatístico de todos os tumores de órbita com diagnóstico anatomopatológico durante o período de janeiro de 1968 a dezembro de 1982, e correlacionada a frequência de tumores de órbita com o número total de tumores diagnosticados neste Laboratório por um período de 5 anos. RESULTADOS: Foram diagnosticados 82 casos de processos expansivos que acometiam a órbita no período estudado, sendo 20,7 por cento do total (17 casos) em crianças (até 14 anos) e os 79,3 por cento restantes (65 casos) em adultos. As crianças apresentaram mais frequentemente gliomas de nervo óptico (4 de 6 casos - 66,6 por cento), retinoblastomas (4 casos - 100 por cento) e rabdomiossarcomas (3 de 4 casos - 75 por cento). Outros diagnósticos menos frequentes em crianças foram meningioma do nervo óptico, neurofibroma, pseudotumor inflamatório, dacrioadenite crônica, neuroma e processo inflamatório crônico. Já a população adulta apresentou maior incidência de carcinomas basocelulares (18 casos), carcinomas epidermóides (12 casos), meningiomas (10 casos), melanomas malignos de coróide (3 casos) e tumores de glândula lacrimal (7 casos). Tumores derivados de estruturas ósseas ou vasculares, pseudotumores e cistos epidermóides intraorbitários foram também diagnosticados, entre outros. De um total de 2.639 tumores diagnosticados neste Laboratório no período de 5 anos (1976 a 1980), foram encontrados 22 tumores orbitários, perfazendo 0,8 por cento do total de casos. CONCLUSÕES: O estudo anatomopatológico destes processos é de fundamental importância para o diagnóstico e para o estabelecimento de terapêuticas adequadas. As ...

PURPOSE: To describe the orbito-sphenoidal expansive processes diagnosed at the Anatomo-Pathological Laboratory of the Fundação Faculdade Federal de Ciências Médicas de Porto Alegre - Complexo Hospitalar Santa Casa de Porto Alegre during a period of 15 years, evaluating their relative frequencies among other tumoral processes. METHODS: We performed a statistical analysis of all orbital tumors with anatomicopathological diagnosis from January 1968 to December 1982, comparing the frequency of orbital tumors with the total number of tumors diagnosed at this Laboratory in a period of 5 years. RESULTS: Eighty-two cases were diagnosed of expansive processes involving the orbit in 15 years - 20.7 percent of the total (17 cases) affecting children (up to 14 years-old) and the remaining 79.3 percent (65 cases) affecting adults. Children presented more frequently optic nerve gliomas (4 of 6 cases 66.6 percent), retinoblastomas (4 cases - 100 percent) and rhabdomiosarcomas (3 of 4 cases - 75 percent). Less frequent diseases in children were optic nerve meningioma, neurofibroma, inflammatory pseudotumor, chronic dacryoadenitis, neuroma and chronic inflammatory process. The adult population presented more cases of basal cell carcinomas (18 cases), squamous cell carciomas (12 cases), meningiomas (10 cases), choroidal malignant melanoma (3 cases) and lacrimal gland tumors (7 cases). Tumors originated from bone or vascular structures, pseudo-tumors, and intraorbital epidermic cysts were also diagnosed, among others. Of a sum of 2,639 tumors diagnosed at this Laboratory in the time period of 5 years (1976 to 1980), there were 22 cases of orbital tumors, reaching a total of 0.8 percent of all cases. CONCLUSIONS: The anatomicopathological study of these processes is somehow important to diagnose and to establish an adequate therapy. The incidence of the expansive processes involving the orbit allows an epidemiological characterization of the different medical ...

Lesões expansivas da órbita: distribuição de casos com estudo histopatológico em 11 anos no Hospital das Clínicas da FMUSP / Orbital space-occupying lesions: an 11-year study of cases with histopathologic analysis seen at Hospital das Clínicas of FMUSP

OBJETIVO: Estudar a incidência das lesões expansivas orbitárias submetidas à biópsia em uma casuística de 11 anos no Hospital das Clínicas da FMUSP, e avaliar quais as principais especialidades envolvidas no diagnóstico e no tratamento de tais afecções. CASUÍSTICA E MÉTODOS: Solicitou-se ao setor de arquivos do hospital todos os casos de tumores e lesões de órbita submetidas à biópsia no período de 1993 a 2004. Dos 924 prontuários obtidos, 115 foram separados após análise dos dados segundo os resultados histopatológicos obtidos. Outros 111 pacientes foram obtidos por meio dos arquivos pessoais da patologista encarregada da Oftalmologia no Departamento de Patologia, dos quais 45 pacientes foram excluídos por coincidirem com a fonte anterior. Os dados foram contabilizados e comparados com os de outros serviços. RESULTADOS: Das 181 lesões orbitárias estudadas, 70 por cento eram primárias, 23 por cento eram secundárias, 6 por cento consistiam de metástases e linfomas e 1 por cento não foi classificada. Em relação à especialidade responsável diretamente pelo paciente, 72,37 por cento dos pacientes foram assistidos por oftalmologistas, 14,36 por cento por cirurgiões de cabeça e pescoço, 6,62 por cento por neurocirurgiões e 6,62 por cento por outros setores do hospital. CONCLUSÕES: As afecções orbitárias são atendidas em sua maioria pelos oftalmologistas especializados em tal área, e ao se incluir nesta casuística outras especialidades, nota-se um aumento no número de casos de lesões tumorais secundárias quando comparada ao de outros trabalhos realizados em serviços no exterior. Há necessidade de criar-se um registro confiável dos casos de lesões expansivas orbitárias nos serviços especializados brasileiros, e confirma-se o caráter multidisciplinar do objeto deste estudo.

PURPOSE: To report the incidence of orbital space-occupying lesions in an 11-year interval study of patients examined at the Hospital das Clínicas of FMUSP, and to evaluate which are the main specialties involved in the diagnosis and treatment of such conditions. METHODS: All cases of orbital space-occupying lesions in the period from 1993 to 2004 were revised. Of 924 obtained medical records, 155 were selected after histopathological analysis. Another 111 cases were gathered from the personal archives of a pathologist responsible for Ophthalmic Histopathology, from which 45 cases were excluded because they had already been included in the first source analysis. The data were analyzed and compared with the results from other institutions. RESULTS: Of the studied 181 orbital space-occupying lesions, 70 percent were primary, 23 percent secondary, 6 percent metastatic and lymphomas, and 1 percent was not classified. The most common specialties involved ophthalmologists in 72.37 percent of all 181 cases, head and neck surgeons in 14.36 percent, neurosurgeons in 6.62 percent, and others in 6.62 percent. CONCLUSIONS: The orbital mass lesions were treated mostly by an expert ophthalmologist. When other specialties were included, there was a modest increase in secondary tumor incidence when the final result was compared with previous studies on this subject. Our findings indicate that the creation of a trustworthy register of all orbital space-occupying lesions in Brazilian specialized services is necessary. The multidisciplinary character of the orbital mass lesions is corroborated by this review.

Tumores intrínsecos de la glándula lagrimal (1976-1996) / Intrinsic tumors of the lachrymal gland

El estudio de 19 pacientes portadores de lesiones tumorales intrínsecas de la glándula lagrimal, atendidos en el Instituto de Oncología y Radiobiología (INOR) en 20 años, reveló que representa el 8,9 por ciento de todas las lesiones tumorales de la órbita. El adenoma pleomórfico (tumor mixto de la glándula) es el más frecuente (42,1 por ciento), seguido de las tumoraciones malignas (36,8 por ciento) y de las lesiones inflamatorias crónicas o dacrioadenitis (21 por ciento). De los pacientes con lesiones malignas, 2 fallecen por progresión de la enfermedad, ambos corresponden al carcinoma adenoideo quístico, lo que reafirma la agresividad de este tipo histológico de tumor. Al analizar los resultados de las técnicas quirúrgicas empleadas encontramos que en 12 pacientes se utilizó orbitotomía superoexterna, craneotomía transfrontal en 6 y la vía transconjuntival en 1, con el 83,3 por ciento, 16,6 por ciento y 100 por ciento, respectivamente, de efectividad (resección total del tumor). En 4 pacientes con adenoma pleomórfico la cirugía seleccionada no fue exerética, donde se observaron recidivas tumorales, 2 de las cuales se transforman en adenocarcinoma, lo que demuestra lo importante de una correcta planificación del tratamietno para reducir los riesgos de recurrencias y transformaciones malignas

Lesiones de la fosa de glándula lagrimal / Injuries of the lachrymal gland fossa

Objetivos: Determinar el tipo de lesiones que ocupan la fosa de la glándula lagrimal en pacientes mayores de 15 años de edad. Material y Método: De un total de 144 pacientes con masas orbitarias se encontraron 42 con masas ubicadas en la región súpero-temporal de la órbita, determinando su naturaleza benigna o no, según el estudio clínico-patológico. Resultados: 22 (52,4 por ciento) lesiones malignas y 20 (47,6 por ciento) lesiones benignas. Entre las malignas, el linfoma no Hodgkin (8 casos) y el carcinoma adenoide quístico (5 casos), fueron los más frecuentes, y entre las benignas, el quiste dermoide (5 casos), el adenoma pleomórfico (4 casos) y la inflamación crónica inespecífica (en 4), fueron las principales. Conclusiones: Si se considera que entre las masas que ocupan espacio en la órbita de nuestros pacientes mayores de 15 años, el 61,8 por ciento son benignas y el 38,2 por ciento son malignas, cuando la masa se ubica en la fosa de la glándula lagrimal la posibilidad de malignidad sube a un 50 por ciento, estamos obligados a estar más alertas frente a este tipo de ubicación orbitaria

Leucemia linfoblástica aguda e envolvimento ocular / Ocular involvement in acute lymphoblastic leucemia

Relato de 3 casos de Leucemia Linfoblástica Aguda com envolvimento ocular. Discorrem a respeito dos modos pelos quais o globo ocular e órbita podem ser envolvidos, seja em consequência das alteraçöes hematológicas inerentes às leucemias ou pela infiltraçäo neoplásica. Comentam acerca do globo ocular como Santuário das Leucemias e relatam complicaçöes terapêuticas que com a evoluçäo da oftalmologia podem ser manejadas com objetivo de melhorar a qualidade de vida destes pacientes