Résumé
Abstract: Background: This systematic review aims to report the current knowledge of retinoblastoma (Rb) and its implications in Mexico. We analyzed clinical and demographic data of patients with Rb at select hospitals with Rb programs or that treat and refer patients with Rb, and identified the gaps in practice. We propose solutions to improve diagnosis, provide adequate treatment, and improve patient uptake. Methods: A general review was conducted on PubMed of peer-reviewed literature on Rb in Mexico. Ophthalmology Department Heads or Directors of Rb programs at seven hospitals in Mexico were contacted for data available on their patients with Rb. Results: Five hospitals provided clinical data on 777 patients with Rb in a period spanning 2000-2015. Of the 122 patients with treatment, 83.4% underwent enucleation. From 33 to 45.3% of Rb tumors in Mexico reach an advanced intraocular stage of development. Knowledge of the disease is limited, despite the fact that the Mexican Retinoblastoma Group has elaborated Rb treatment guidelines and is developing a national Rb registry. Especially in the Southern states, prevalence and outcomes are comparable to African and Asian countries, and only few patients are referred to national treatment centers. Only three institutions have comprehensive Rb programs. Conclusions: There is an immediate need in Mexico to expand primary care providers' knowledge of Rb and to expand and upgrade current Rb programs to meet the needs of the population adequately. Diagnosis and care of Rb patients in Mexico can also be improved by the establishment of a national Rb registry and a national early detection program, and by increased use of the national treatment protocol.
Resumen: Introducción: Esta es una revisión sistemática de los conocimientos actuales del retinoblastoma (Rb) y sus implicaciones en los centros de referencia más importantes del país. Se presenta un análisis situacional de los programas de Rb en México, se identificaron las brechas en la práctica, y se proponen soluciones para mejorar el diagnóstico, tratamiento y referencia oportuna de pacientes. Métodos: Se realizó una revisión general de la literatura publicada sobre Rb en México a través de PubMed. Los datos sociodemográficos de pacientes con Rb fueron obtenidos a través de los directores de programas de retinoblastoma en siete hospitales. Resultados: Casi una tercera parte de los casos Rb se diagnostican en estadios avanzados. A pesar de la existencia del Grupo Mexicano de Retinoblastoma, el conocimiento de esta patología entre los médicos es limitado. Las diferencias en el tratamiento son notorias en el sur del país, donde la prevalencia y los resultados son comparables con África y Asia. Solamente tres instituciones a nivel nacional tienen un programa establecido de Rb. Conclusiones: Existe la necesidad inmediata de consolidar los programas de Rb para cubrir las necesidades reales de la población. Se requiere mejorar la educación del médico de primer contacto, establecer el registro nacional de casos y el programa de detección temprana, establecer los programas de salvamento ocular nacional, y reforzar las instituciones que brindan tratamiento.
Sujets)
Humains , Rétinoblastome/diagnostic , Guides de bonnes pratiques cliniques comme sujet , Tumeurs de la rétine/diagnostic , Orientation vers un spécialiste/statistiques et données numériques , Rétinoblastome/thérapie , Rétinoblastome/épidémiologie , Enregistrements , Connaissances, attitudes et pratiques en santé , Prévalence , Tumeurs de la rétine/thérapie , Tumeurs de la rétine/épidémiologie , Dépistage précoce du cancer/méthodes , Mexique/épidémiologieRésumé
A 4 year-old girl with bilateral, non-familial retinoblastoma (RB) was referred to our care after primary enucleation OS and active tumor OD refractory to multiple therapies (intravenous chemotherapy, laser/cryotherapy, and I-125 plaque radiotherapy). Vitreous seeding OD, initially controlled by several sessions of Ophthalmic Artery Infusion Chemotherapy (OAIC) and periocular chemotherapy, recurred shortly thereafter. The patient underwent intravitreal (IVit) Melphalan injections achieving tumor control despite the concurrent development of keratopathy, pupillary synechiae, cataract, and necrosis of the inferior fornix and the adjacent orbital fat, all secondary to the treatments administered. Repeated amniotic membrane implants and tarsorrhaphy were performed to alleviate the symptoms. Despite being tumor free for 6 months, a poor fundus view and treatment-related complications prompted us to consider enucleation, but parents declined. Following recent negative magnetic resonance imaging (MRI), her cataract was removed. She was then found to have tumor recurrence. Her eye was enucleated 12 months ago and she recovered well from the surgery. As ocular oncology embarks in eye-preserving treatments for retinoblastoma, it is important to address the cumulative effects and associated impact of such treatments and the possibility of failure.
Uma menina de 4 anos com retinoblastoma (RB) bilateral, não-familiar foi encaminhada após enucleação OE e tumor ativo OD refratário a múltiplas terapias (quimioterapia endovenosa, laser/crioterapia e braquiterapia com I-125). Semeadura vitrea OD, inicialmente controlada por inúmeras sessões de Quimioterapia Intra-Arterial Oftálmica (QIAO) e quimioterapia periocular, recorreu em seguida. Paciente recebeu injeções intravítreas de Melphalan obtendo controle tumoral apesar do desenvolvimento concomitante de ceratopatia, sinéquias pupilares, catarata, necrose do fórnice inferior e gordura periorbitária adjacente, todos secundários aos tratamentos usados. Implantes repetidos de membrana amniótica e tarsorrafias foram realizadas para melhora sintomatológica. Apesar de estar livre de tumor por 6 meses, a baixa visibilidade do fundo e complicações terapêuticas nos levaram a considerar enucleação que foi descartada pelos pais. Após recente ressonância magnética nuclear (RMN) negativa, a catarata foi removida. Foi então detectada recorrência tumoral. O olho foi enucleado há 12 meses e ela se recuperou bem da cirurgia. Enquanto a oncologia ocular embarca em tratamentos para preservar em retinoblastoma, é importante considerar os efeitos cumulativos e impacto associado desses tratamentos, e a possibilidade de fracasso.
Sujets)
Enfant d'âge préscolaire , Femelle , Humains , Récidive tumorale locale/thérapie , Tumeurs de la rétine/thérapie , Rétinoblastome/thérapie , Association thérapeutique/effets indésirables , Association thérapeutique/méthodes , Énucléation oculaireRésumé
Objetivo: reporte de casos que han presentado quistes retinianos maculares de fisiopatología traccional y con implicaciones ulteriores, sea hacia la involución espontánea o la progresión hacia agujero macular y su manejo. Métodos: estudio observacional descriptivo, tipo serie de casos, observados por retinólogos de una Institución Oftalmológica, estudiados en seguimiento y documentados con tomografía óptica coherente espectral. Resultados: fueron satisfactorios anatómica y funcionalmente según su evolución y manejo. Conclusiones: los quistes maculares traccionales pueden involucionar favorablemente o conducir a agujeros de manejo quirúrgico y pronóstico variable.
Purpose: to report cases presenting tractional macular cysts and subsequent implications either spontaneous involution or progression to macular holes and their management. Methods: retrospective study of case series observed by retinologists in a general ophthalmological institution, evaluated on follow-up and documented with spectral domain optical coherente tomography. Results: satisfactory, both anatomically and functionally according with evolution and management. Conclusions: tractional macular cysts may tend to a favorable spontaneous involution or progression to macular holes that require surgical management of variable prognosis.
Sujets)
Tumeurs de la rétine/thérapie , Perforations de la rétine/chirurgie , Perforations de la rétine/physiopathologie , Tumeurs de la rétine/physiopathologieRésumé
We describe the case of a 9-month old boy with unilateral retinoblastoma and bulftalmo. Primary enucleation was the treatment of choice due to the lack of visual prognosis. The histology of the enucleated eye showed massive choroidal invasion by the tumor and the optic nerve free of neoplastic tissue. Therefore, no adjuvant chemotherapy or radiotherapy was indicated. Three months after the enucleation, the patient returned with massive orbital retinoblastoma with exposure of the conjunctiva. Treated with chemotherapy, the patient has been in remission for 12 months. The risk factors for orbital recurrence are discussed.
Relatamos paciente de 9 meses de idade com buftalmo e retinoblastoma unilateral. O paciente foi tratado com enucleação primária devido à falta de prognóstico visual. O exame do olho enucleado mostrou invasão maciça de coroide e ausência de comprometimento do nervo óptico não sendo, portanto, submetido a tratamento adjuvante de quimioterapia ou de radioterapia. Três meses após a enucleação, o paciente apresentou-se com retinoblastoma orbitário volumoso com exposição da conjuntiva. Tratado com quimioterapia permanece em remissão após 12 meses. São discutidos os fatores de risco para a ocorrência da recidiva orbitária.
Sujets)
Humains , Nourrisson , Mâle , Tumeurs de l'orbite , Tumeurs de la rétine , Rétinoblastome , Énucléation oculaire , Récidive tumorale locale/anatomopathologie , Récidive tumorale locale/thérapie , Tumeurs de l'orbite/anatomopathologie , Tumeurs de l'orbite/thérapie , Facteurs de risque , Tumeurs de la rétine/anatomopathologie , Tumeurs de la rétine/thérapie , Rétinoblastome/anatomopathologie , Rétinoblastome/thérapie , Résultat thérapeutiqueRésumé
Retinal vasoproliferative tumor is a rare disease that has capillary hemangioma as the most frequent diferential diagnosis. The tumor is considered to be of reactive nature. It can be idiophatic or secondary to other ocular diseases such as: uveitis, retinitis pigmentosa, sickle cell disease, previous surgery and retinopathy of prematurity. Lesions with no exsudation or visual decrease can be observed. Lesions that need treatment can be managed by on or more modalities such as cryotherapy, a variety of lasers, surgical excision, radiation, and antiangiogenic intravitreal injections.
O tumor vasoproliferativo da retina é uma lesão rara, cujo principal diagnóstico diferencial é o hemangioma capilar da retina. O tumor tem natureza reacional. Pode ser idiopático ou secundário a outras doenças como: uveítes, retinose pigmentar, retinopatia da anemia falciforme, cirurgia prévia e retinopatia da prematuridade. Lesões sem exsudação ou baixa visual podem ser observadas. Quando há indicação de tratamento este pode ser feito pela crioterapia, vários tipos de lasers, excisão cirúrgica, radioterapia e injeções intravítrea de antiangiogênicos, isoladamente ou em associação.
Sujets)
Humains , Tumeurs de la rétine/diagnostic , Tumeurs de la rétine/thérapie , Néovascularisation rétinienne/diagnostic , Néovascularisation rétinienne/thérapie , Curiethérapie , Cryothérapie , Diagnostic différentielRésumé
Retinoblastoma is a rare malignancy of the retina seen exclusively in children. It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed. We report a case in a five-day-old infant in whom treatment (chemotherapy) was delayed by a month due to high bilirubin levels secondary to physiological jaundice, which gave us the unique opportunity to measure the growth of the tumor over a month. This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.
Sujets)
Antinéoplasiques/administration et posologie , Protocoles de polychimiothérapie antinéoplasique , Carboplatine/administration et posologie , Évolution de la maladie , Association de médicaments , Étoposide/administration et posologie , Études de suivi , Fond de l'oeil , Humains , Hyperthermie provoquée , Nouveau-né , Ictère/complications , Tumeurs de la rétine/complications , Tumeurs de la rétine/anatomopathologie , Tumeurs de la rétine/thérapie , Rétinoblastome/complications , Rétinoblastome/anatomopathologie , Rétinoblastome/thérapie , Résultat thérapeutique , Vincristine/administration et posologieRésumé
Objective. To evaluate compliance to treatment in advanced retinoblastoma. Methods. This is a retrospective descriptive study of patients of retinoblastoma registered at our cancer center from June 2003-February 2007 to study compliance. Results. Sociodemographic data of 177 patients revealed rural:urban ratio of 2:1, median age 36 months (unilateral disease) and 24 months (bilateral disease); median symptom duration 7.2 months. Overall, 84/141 evalued cases took adequate therapy; follow-up could be achieved in 67/141 (47.5%) cases in comparison to 92.4% in Hodgkin’s lymphoma (p=0.001), 62.8% in acute myeloid leukemia (p=0.036) and 72.7% in non Hodgkin’s lymphoma (p=0.001). There was no significant impact of any sociodemographic factors on compliance. Amongst those offered enucleation upfront for intraocular disease, it was accepted in 86/93 (92.5%) eyes. Conclusions. This is one of the largest studies in relation to sociodemographic factors and clinical spectrum, and the only study from Asia evaluating compliance with recommended therapy in retinoblastoma. In order to improve ocular and patient salvage rates in Asian countries, exact causes for poor compliance in retinoblastoma need to be closely examined through a prospective study.
Sujets)
Adolescent , Enfant , Enfant d'âge préscolaire , Démographie , Femelle , Humains , Inde/épidémiologie , Nourrisson , Mâle , Observance par le patient , Enregistrements , Tumeurs de la rétine/épidémiologie , Tumeurs de la rétine/thérapie , Rétinoblastome/épidémiologie , Rétinoblastome/thérapie , Études rétrospectivesRésumé
A 3-year-old girl presented with left exotropia. Funduscopy demonstrated a retinocytoma associated with five discrete retinoblastomas in the left eye and three discrete retinoblastomas in her right eye. The clinical manifestations and fundus imaging findings are described.
Sujets)
Enfant d'âge préscolaire , Femelle , Humains , Tumeurs primitives multiples/anatomopathologie , Tumeurs primitives multiples/thérapie , Tumeurs de la rétine/anatomopathologie , Tumeurs de la rétine/thérapie , Rétinoblastome/anatomopathologie , Rétinoblastome/thérapieRésumé
Relatamos cinco casos de retinocitoma diagnósticados em um período de 75 meses. A média de idade ao diagnóstico foi de 193,2 meses. Dois casos apresentavam tumor unilateral, um bilateral e dois exibiam retinoblastoma no olho contralateral. As características clínicas e oftalmoscópicas, as implicações genéticas e a necessidade de tratamento e acompanhamento contínuo dos pacientes com retinocitoma são discutidas.
Five retinocytoma cases diagnosed over 75 months are reported. The mean age at diagnosis was 193.2 months. Two cases were unilateral, one was bilateral and two exhibited retinoblastoma in the fellow eye. Clinical and ophthalmoscopic aspects, genetical implications and the need of treatment and continuous follow-up of retinocytoma patients are discussed.
Sujets)
Adulte , Enfant d'âge préscolaire , Femelle , Humains , Mâle , Jeune adulte , Tumeurs de la rétine/anatomopathologie , Rétinoblastome/anatomopathologie , Calcinose/anatomopathologie , Tumeurs de la rétine/thérapie , Rétinoblastome/thérapie , Jeune adulteRésumé
El retinoblastoma comprende el 4 por ciento de tumoraciones malignas en edad pediátrica con una incidencia de 1 por cada 20000 niños vivos y en USA se presenta por año entre 250-300 niños, 50-95 por ciento sobreviven a nivel mundial, no hay predisposición por sexo o raza y entre ellos 25-35 por ciento se presentan en forma bilateral, la edad aproximada para el diagnóstico varia en las formas unilaterales alrededor de los 12 meses hasta las formas bilaterales a los 24 meses. Preescolar femenino de 4 años de edad natural de Socopó, procedente de El Piñal quien inicia enfermedad actual de 2 años de evolución caracterizada por la presencia de masa tumoral en ojo izquierdo la cual se incrementa en los últimos 4 meses llegando hasta la protrusión del globo ocular y de 6 días limitación para la oclusión, rubor en párpado superior izquierdo y fiebre. Asimetría ocular, protrusión del globo ocular izquierdo, limitación para la apertura, hifema y no se visualiza córnea, pupilas ni el iris. Es evaluada por el servicio de Oftalmología: OI: Blefarospasmo reactivo, eritema bipalpebral y edema que limitan la apertura ocular. Biomicrooscopia: Quemosis conjuntival en 360 con salida de secreción purulenta a nivel temporal de la conjuntiva, pannus corneal periférico en banda cálcica, opacidad de medios transparentes. LDH 267 UI/I. Ultrasonido Ocular: globo ocular izquierdo: faquico, con lesión sólida localizada en pared ocular extendiéndose anteriormente, sin movilidad, con ecos de alta reflectividad en su interior con sombrado posterior de pared ocular. TAC de cráneo y órbita: imagen radiólucida que infiltra globo ocular izquierdo sin compromiso del nervio óptico, resto sin anormalidad, TAC de tórax y abdomen: normal, Ecosonograma Abdominal: normal, Gammagrama Óseo: normal. Se realizó exanteración de la órbita del globo ocular izquierdo. Biopsia que microscópicamente evidencia tumor infiltra todas las capas con microscopia donde evidencia pseudoresetas en masa tumoral.
Sujets)
Humains , Femelle , Enfant d'âge préscolaire , Exophtalmie/anatomopathologie , Tumeurs de la rétine/diagnostic , Tumeurs de la rétine/anatomopathologie , Tumeurs de la rétine/thérapie , Phénomènes physiologiques oculaires , Résultat thérapeutiqueRésumé
OBJECTIVE: To present the characteristics and treatment outcomes of patients with retinoblastoma. METHODS: A retrospective case series was conducted to review the records of all new patients diagnosed with retinoblastoma between 2003 and 2005. Eyes with early disease, or advanced disease with potential vision were treated with chemotherapy (carboplatin and etoposide) in conjunction with early local therapy (laser or cryo). Radiotherapy was used in cases where the disease did not respond to the above protocols or in recurrent cases. Eyes in the late stage of disease with no potential vision in the initial examination or eyes and where conservative treatment had failed were enucleated. RESULTS: In total, we reviewed 28 new cases of retinoblastoma, 15 of which were unilateral and 13 of which were bilateral (46 percent). These data correspond to a mean of 9.3 new cases per year (0.77 case/ month). The mean age at diagnosis was 33.8 months for unilateral cases, and 19.15 months for bilateral cases (p=0.015). Leucocoria was the major presenting symptom (75 percent). All but one patient with unilateral disease had the affected eye enucleated due to advanced disease (mean follow-up: 39.91 months). Among the 13 bilateral cases, 13 eyes (50 percent) were enucleated, 11 eyes (42.4 percent) were saved with chemotherapy in conjunction with local therapy and 2 eyes (7.6 percent) were saved using external beam radiotherapy (mean follow-up: 41.91 months). In unilateral and bilateral disease, pathology data revealed choroid involvement in 50 percent and 30 percent, respectively, and optic nerve invasion in 92 percent and 50 percent, respectively. CONCLUSION: In this population, retinoblastoma was diagnosed too late and most eyes were consequently enucleated. In cases with bilateral disease, half of the eyes were preserved.
Sujets)
Enfant d'âge préscolaire , Femelle , Humains , Nourrisson , Mâle , Tumeurs de la rétine/thérapie , Rétinoblastome/thérapie , Brésil , Méthodes épidémiologiques , Hôpitaux universitaires , Tumeurs de la rétine/diagnostic , Rétinoblastome/diagnosticRésumé
Methods: Retrospective analysis of clinical charts of 41 children (59 eyes) diagnosed with retinoblastoma and treated by a multidisciplinary team at Hospital Luis Calvo Mackenna in Santiago-Chile, between 1999 and 2007. The information included gender, laterality, diagnosis age, presenting signs, tumor spread, treatment modality and survival rate. Results: A total of 23 cases (56 percent) were unilateral and 18 cases (44 percent) were bilateral. The mean age at diagnosis was 21.6 months (range 2 - 84) and 27 children (65.9 percent) were male. The most common presenting signs were leucokoria (51.2 percent), strabismus (24.4 percent) and proptosis (4.9 percent). Enucleation was performed in 48 eyes (81.3 percent), being the only required treatment in 17 children (41.5 percent). The remaining 24 patients received systemic and/or local therapy with chemotherapy, focal therapy and external beam radiation. 5 children died during the follow - up study period, due to extraocular extension to the orbit, central nervous system and bone marrow. Conclusion: In spite of high enucleation rate as initial therapy for retinoblastoma, the survival rate with this current treatment protocol is similar to those from developed countries.
Se presenta un estudio retrospectivo de las fichas clínicas de 41 niños (59 ojos) con diagnóstico de retinoblastoma tratados por un equipo multidisciplinario en el Hospital Luis Calvo Mackenna, Santiago, Chile, entre los a±os 1999-2007. Se recolectó información respecto al género, edad al diagnóstico, signos de presentación, lateralidad, diseminación del tumor, tipos de tratamiento y sobrevida. Veintitrés casos (56 por ciento) fueron unilaterales y 18 (44 por ciento) bilaterales. La edad promedio al momento del diagnóstico fue de 21,6 meses (rango 2-84) y 27 niños (65,9 por ciento) fueron hombres. Los signos de presentación más frecuentes fueron leucocoria (51,2 por ciento), estrabismo (24,4 por ciento) y proptosis (4,9 por ciento). Se realizó enucleación en 48 ojos afectados (81,3 por ciento), siendo el único tratamiento necesario en 17 niños (41,5 por ciento). Los 24 pacientes restantes recibieron tratamientos complementarios locales y/o sistémicos en la modalidad de quimioterapia, terapia focal y radioterapia externa. Durante el período de seguimiento del estudio fallecieron 5 niños, todos ellos con extensión extraocular de la enfermedad hacia la órbita, sistema nervioso central o médula ósea. Conclusiones: No obstante el alto porcentaje de pacientes con retinoblastoma que requieren enucleación como terapia inicial, la tasa de sobrevida con el protocolo actual de tratamiento es comparable a la de países desarrollados.
Sujets)
Humains , Mâle , Femelle , Nourrisson , Enfant d'âge préscolaire , Enfant , Tumeurs de la rétine/épidémiologie , Tumeurs de la rétine/thérapie , Rétinoblastome/épidémiologie , Rétinoblastome/thérapie , Association thérapeutique , Chili/épidémiologie , Énucléation oculaire , Études de suivi , Tumeurs de la rétine/classification , Tumeurs de la rétine/mortalité , Tumeurs de la rétine/anatomopathologie , Études rétrospectives , Rétinoblastome/classification , Rétinoblastome/mortalité , Rétinoblastome/anatomopathologie , Taux de survieRésumé
É relatado um caso de retinoblastoma de aparecimento tardio, com características pouco freqüentes, com o objetivo de melhorar o controle da afecção. Em 1993, SJMMF, nove meses, masculino, leucodermo, apresentou tumor na retina esquerda. O paciente tinha história familiar de retinoblastoma. A enucleação foi realizada, comprovando-se retinoblastoma. Os exames para estadiamento sistêmico foram normais. O olho direito evoluiu normal por dez anos. Em exame de rotina, foram constatadas três lesões de retinoblastoma na retina nasal. Foi feita termoterapia transpupilar, com laser de diodo de 810 nm. Após 30 dias, as lesões regrediram. Após 60 dias houve recidiva na borda da lesão, onde foi realizada crioterapia transescleral, com regressão do tumor por seis meses. Durante o controle, observaram-se condensações próximas à lesão tumoral atrófica (sementes vítreas). Foi feito braquiterapia com Iodo125, havendo desaparecimento das mesmas após 30 dias. Novas sementes surgiram três meses pós-braquiterapia, depositadas na superfície retiniana, sendo tratadas com crioterapia transescleral e termoterapia transpupilar, havendo regressão. O paciente evoluiu com nova semente vítrea após seis meses, a qual, após depositar-se na superfície da retina, foi tratada com termoterapia transpupilar. Está em seguimento há 38 meses desde o aparecimento do tumor bilateral, mantendo acuidade visual de 20/20 e exames clínicos normais. Considera-se importante este caso pela pouca freqüência de aparecimento da doença nesta idade. Julga-se necessário o alerta para os casos de retinoblastoma já considerados curados.
A case of retinoblastoma with uncommon features is reported, aiming at improving follow-up. In 1993, SJMMF, 9-month-old white boy, presented a squint in the left eye. A retinal tumor was detected. The patient had a family history of retinoblastoma. Enucleation was performed and retinoblastoma was proved. The patient underwent examination for staging, all were normal. The fellow eye remained normal for 10 years. During routine examination the retina map revealed three retinoblastoma white lesions in the nasal retina. The patient underwent transpupillary thermotherapy with diode laser. After 30 days, the lesions became atrophic. After 60 days there was tumor recurrence on the border of the lesion. Sclera cryotherapy was performed. There was tumor regression for six months. During follow-up, condensations next to the atrophic tumor lesions were discovered, which were vitreous seeds. Brachitherapy with 125 iodine was done. The lesions disappeared after 30 days. New vitreous seeds appeared 3 months later, set on the retina surface. They were blocked with sclera cryotherapy and transpupillary thermotherapy. The patient presented with new vitreous seeds after six months, which adhered to the retina. They were blocked with transpupillary thermotherapy. Follow-up was for 38 months since the appearance of the bilateral tumor. The patient presents normal visual acuity and clinical examination. This case is important considering the low frequency of the disease at this age. It is essential to maintain alertness when cases of retinoblastoma are seen as cured.
Sujets)
Humains , Nourrisson , Mâle , Récidive tumorale locale , Tumeurs de la rétine , Rétinoblastome , Curiethérapie , Cryothérapie , Énucléation oculaire , Thérapie laser , Récidive tumorale locale/diagnostic , Récidive tumorale locale/thérapie , Reprise du traitement , Tumeurs de la rétine/diagnostic , Tumeurs de la rétine/chirurgie , Tumeurs de la rétine/thérapie , Rétinoblastome/diagnostic , Rétinoblastome/chirurgie , Rétinoblastome/thérapieRésumé
OBJETIVO: Descrever um caso de tumor vasoproliferativo associado à tuberculose ocular tratado com crioterapia e injeção de triancinolona intravítrea. DESENHO DO ESTUDO/PACIENTES E MÉTODOS: Relato de caso intervencional. Paciente do sexo feminino, 42 anos, referia inflamação do olho direito havia 1 ano, não diagnosticada, tratada com prednisona oral por 30 dias. Relatava baixa visão com o olho direito (OD) desde a infância. Negava antecedentes pessoais e familiares. Ao exame ocular, apresentava acuidade visual menor que 20/400 com o OD. Não apresentava alterações à biomicroscopia e a pressão intra-ocular era normal em ambos os olhos. A fundoscopia do OD mostrava lesão vascularizada, elevada, associada ao descolamento seroso e exsudatos duros, localizada na periferia inferior da retina. A lesão era cercada por extensa área de pontos de hiperplasia do epitélio pigmentar. A mácula apresentava diminuição do reflexo foveal. No exame de ultra-som ocular, a lesão apresentava altura igual a 2,25mm e consistência sugestiva de lesão vascularizada. Foram solicitados exames sorológicos, hemograma, RX tórax e PPD. Os exames foram normais, com exceção do PPD, considerado forte reator. A paciente foi encaminhada ao infectologista, que diagnosticou tuberculose após exame de pesquisa de BK no escarro. Em face do quadro clínico, foi feito o diagnóstico de tumor vasoproliferativo da retina associado à tuberculose ocular presumida. Iniciou-se o tratamento com esquema tríplice (rifampicina, isoniazida e piridoxina). Optou-se por tratar o tumor com crioterapia e injeção intravítrea de triancinolona (4 mg/ml). Após 30 dias, a paciente apresentava diminuição do descolamento seroso e áreas atróficas na lesão tumoral. O aspecto angiofluoresceinográfico mostrava algumas áreas de enchimento precoce da trama vascular com discreto extravasamento tardio do contraste, sem áreas de oclusão capilar. A maior parte da lesão apresentava hipofluorescência por bloqueio (proliferação...
PURPOSE: To describe a case of vasoproliferative tumor associated with presumed ocular tuberculosis treated with cryotherapy and intravitreal triamcinolone injection. STUDY DESIGN/PATIENT AND METHODS: Interventional case report. A 42-year-old female patient reported inflammation of the right eye 1 year ago, treated with oral prednisone for 30 days. She referred blurred vision in the right eye since childhood. Ophthalmologic examination showed 20/400 visual acuity in the right eye. Biomicroscopy and intraocular pressure were normal. Fundus examination showed vascularized elevated lesion, associated with serous retinal detachment and hard exudates at the inferior periphery. The lesion was surrounded by extensive area of hyperplastic retinal pigment epithelium. The macula showed attenuation of the foveal reflex. Ocular ultrasound showed a 2.25 mm height vascularized lesion. Serologic examination, hemogram and thorax RX were normal. PPD was considered strong reactor and tuberculosis was diagnosed after positive BK research. Considering the clinical aspects, the ocular diagnosis was vasoproliferative tumor associated with presumed ocular tuberculosis. Treatment with rifampicin, isoniazide and pyridoxine was started. We decided to treat the ocular tumor with cryotherapy and intravitreal triamcinolone injection (4 mg/ml). After 30 days, serous detachment was smaller and the tumor showed atrophic areas. Fluorescein angiography showed areas of vascular hyperfluorescence with slight extravasation and areas of blocked fluorescence due to RPE hyperplasia. Secondary vasoproliferative tumors are retinal glial proliferations and are associated with many ocular conditions that affect retina and choroid. Because of the great number of associated complications, with important visual loss, vasoproliferative tumors should be treated at the moment of diagnosis. Treatment of choice is cryotherapy. Intravitreal triamcinolone can be used as adjuvant treatment...
Sujets)
Adulte , Femelle , Humains , Hémangiome/étiologie , Tumeurs de la rétine/étiologie , Tuberculose oculaire/complications , Antituberculeux/usage thérapeutique , Cryothérapie , Glucocorticoïdes/usage thérapeutique , Hémangiome/diagnostic , Hémangiome/thérapie , Tumeurs de la rétine/diagnostic , Tumeurs de la rétine/thérapie , Triamcinolone/usage thérapeutique , Tuberculose oculaire/diagnostic , Tuberculose oculaire/traitement médicamenteuxRésumé
OBJETIVO: Descrever e analisar os principais achados histopatológicos no retinoblastoma com ênfase na invasão de nervo óptico e túnicas oculares, correlacionando-os ao tratamento realizado. MÉTODOS: Vinte e oito olhos com diagnóstico de retinoblastoma tratados por enucleação na Universidade Federal de São Paulo entre dezembro de 2000 e outubro de 2002 foram submetidos à revisão histopatológica. Os dados clínicos incluiram idade, sexo, raça, lateralidade e tratamento realizado. Na revisão histopatológica foram avaliados neovascularização da íris e seio camerular, grau de diferenciação do tumor e invasão do nervo óptico e coróide de acordo com a classificação de Khelfaoui. RESULTADOS: De 27 pacientes, 13 (48,1 por cento) eram do sexo masculino e 14 (51,9 por cento) eram do sexo feminino, 16 (59,3 por cento) eram da raça branca, 5 (18,5 por cento) eram da raça amarela, 4 (14,8 por cento) eram pardos e 2 (7,4 por cento) eram negros. A média da idade dos pacientes foi de 22,7 meses, sendo 13 casos bilaterais e 14 casos unilaterais. Com relação ao tratamento utilizado, 13 (46,4 por cento) olhos foram submetidos a enucleação primária e 15 (53,6 por cento) tiveram tratamento prévio. Na avaliação histopatológica, 15 (53,6 por cento) tumores eram bem diferenciados. Neovascularização da íris e/ou seio camerular foram observados em 60,6 por cento e necrose e calcificação em 89,2 por cento dos casos. O envolvimento da coróide foi observado em 18 (64,2 por cento) dos casos (grau II e III) e a invasão de nervo óptico em 8 (28,5 por cento) (graus II, III e IV). Dos casos submetidos ao tratamento prévio 40 por cento tinham algum grau de invasão das túnicas oculares e apenas 6,67 por cento apresentavam simultaneamente invasão do nervo óptico e das túnicas oculares. Entretanto nos olhos que foram submetidos à enucleação primária 30,7 por cento apresentavam invasão das túnicas oculares e 53,8 por cento apresentavam simultaneamente invasão do nervo óptico e das túnicas oculares. CONCLUSAO: Neovascularização, necrose e calcificação foram achados mais comumente observados. A invasão do nervo óptico e coróide foram os dois fatores prognósticos mais importantes, encontrados em 28,5 por cento e 64,2 por cento dos casos respectivamente. Os casos tratados com enucleação primária apresentavam maior comprometimento ocular.
Sujets)
Humains , Mâle , Femelle , Nourrisson , Enfant d'âge préscolaire , Enfant , Adolescent , Adulte , Adulte d'âge moyen , Sujet âgé de 80 ans ou plus , Choroïde/anatomopathologie , Tumeurs de la rétine/anatomopathologie , Nerf optique/anatomopathologie , Rétinoblastome/anatomopathologie , Association thérapeutique , Énucléation oculaire , Invasion tumorale/anatomopathologie , Tumeurs de la rétine/thérapie , Néovascularisation pathologique/anatomopathologie , Pronostic , Rétinoblastome/thérapieRésumé
Os autores relatam um caso de retinoblastoma bilateral, olho direito com prognóstico muito desfavorável, submetido a enucleaçäo e diagnosticado regressäo espontânea pela anatomia patológica. Olho esquerdo com prognóstico desfavorável, tratado conservadoramente com radioterapia convencional e quimioterapia evoluindo com preservaçäo da visäo. Comentam acerca das modalidades de regressäo do retinoblastoma, espontânea e terapêutica, e como estes pacientes devem ser acompanhados, visando recorrências, visando o aparecimento de novos focos tumorais e a ocorrência de outras neoplasias malígnas primárias.