Evaluación de secuelas visuales y cosméticas del tratamiento conservador con terapia radiante externa en pacientes con retinoblastoma bilateral en el Hospital de Pediatría Garrahan / Assessment of visual and cosmetic sequelae of conservative treatment of external beam radiation of patients with bilateral retinoblastoma at the Pediatric Hospital Garrahan

El Retinoblastoma es la neoplasia ocular más frecuente en pediatría. La Terapia radiante externa fue hasta hace una década el tratamiento conservador de elección. Luego se incluyó la quimio reducción; en un intento de evitar la radioterapia externa y sus complicaciones. En este estudio retrospectivo evaluamos los resultados del tratamiento conservador con terapia radiante externa o con quimio reducción en el servicio de oftalmología del Hospital Nacional de Pediatría Juan P. Garrahan, desde 1987 a 2009. De un total de 571 pacientes con diagnóstico de Retinoblastoma, 341 fueron unilaterales y 217 bilaterales. De estos últimos se analizaron 166 pacientes cuya edad media al diagnostico fue de 12 meses. Se trataron 332 ojos de 166 pacientes con Retinoblastoma bilateral, 157 ojos (47,3%) recibieron quimioreducción como tratamiento inicial, 115 ojos (34,6%) fueron enucleados al inicio, 45 ojos (13,6%) recibieron radioterapia externa como único tratamiento y 15 ojos (4,5%) recibieron tratamiento local solo (laser o crioterapia) como primera elección. Se analizaron los datos con el programa estadístico STATA 12.0 stataCorp Texas.USA. La agudeza visual final fue superior a 20/70 en el 51,5% de los pacientes e inferior en el 48,5%. Se encontró una relación significativa (p=0,005) entre el estadio al diagnóstico y la agudeza visual final; los pacientes con discapacidad visual se presentaron con estadios avanzados. Se evaluaron todas las orbitas enucleadas (157); de ellas el 74,5% recibieron radioterapia externa antes o después de la enucleación. El 24,8% de las orbitas irradiadas presentaron deformidad de la cavidad, con mala adaptación de prótesis y retracción orbitaria, el 70,1% presentaron cambios que permitían una adaptación de prótesis aceptable con alguna limitación de movilidad y solo 6 orbitas (5,12%) presentaban una muy buena cavidad para adaptación cosmética. Cuarenta de 157 orbitas enucleadas no recibieron radioterapia en ningún momento (25,5%), el 92,5% de ellas presentaron buena adaptación y solo el 7,5% tuvieron problemas de adaptación debido a complicaciones postoperatorias. El diagnóstico precoz, el tratamiento oportuno, y el uso de quimio reducción como terapia inicial en Retinoblastoma intraocular, permiten aumentar la tasa de preservación del globo ocular y reducen o eliminan la necesidad de recibir Terapia radiante externa, evitando sus secuelas (AU)

Retinoblastoma is the most common ocular neoplasia in childhood. External beam radiation therapy was the conservative treatment of choice until a decade ago. Subsequently, chemoreduction was added trying to avoid external beam radiation therapy and its complications. In this retrospective study we assess the results of conservative therapy with external beam radiation therapy or with chemoreduction at the Department of Ophthalmology at the Pediatric Hospital Juan P. Garrahan between 1987 and 2009. Of a total of 571 patients with a diagnosis of retinoblastoma, 341 had unilateral and 217 bilateral retinoblastoma. Of the latter patients, 166 patients were analyzed with a mean age at diagnosis of 12 months. Overall, 332 eyes of 166 patients with bilateral retinoblastoma were treated; at initial treatment 157 eyes (47.3%) underwent chemoreduction, 115 eyes (34.6%) were enucleated, 45 eyes (13.6%) underwent external beam radiation therapy as the only treatment, and 15 eyes (4.5%) only received local treatment (laser or cryotherapy) as a first choice. Data were analyzed using STATA 12.0 stataCorp Texas.USA. Final visual acuity was more than 20/70 in 51.5% and less in 48.5% of the patients. A significant relationship (p=0.005) between stage at diagnosis and final visual acuity was found; patients with visual impairment presented with advanced stages. All enucleated orbits were assessed (157); 74.5% underwent external beam therapy before or after enucleation. Of all irradiated orbits, 24.8% presented with cavity deformity, poor prosthesis fit, or contraction of the socket. Of all patients, 70.1% presented with changes that allowed acceptable fitting of the prosthesis with slight movement limitation and only 6 orbits (5.12%) had a good cavity for cosmetic appearance. Forty of 157 enucleated orbits did not receive radiation therapy at any moment (25.5%); 92.5% of them had a good fitting and in only 7.5% fitting problems due to postoperative complications were found. Early diagnosis, adequate treatment, and use of chemoreduction as initial therapy of intraocular retinoblastoma allow for an increased rate of preservation of the eye and reduce or eliminate the need for external beam therapy and its sequelae (AU)

Diagnóstico y tratamiento de retinoblastoma / Diagnosis and treatment of retinoblastoma

El retinoblastoma es el tumor maligno más frecuente en la infancia, sin embargo sigue siendo un tumor raro en niños. Se asocia a una inactivación de ambos alelos del gen supresor tumoral. Pueden aparecer esporádicamente o pueden ser familiares y existen varios patrones de diseminación tumoral que pueden variar el pronóstico tanto visual como del globo ocular. El signo más frecuente es la leucocoria seguido por estrabismo, con una edad promedio de presentación a los 2 años en casos unilaterales y durante del primer año de vida en los tumores bilaterales. La detección temprana y tratamiento adecuado permite una mayor sobrevida en el niño.

Retinoblastoma is the most common malignant tumor in childhood; however it is ar are tumor in children. It is associated with inactivation of both alleles of the tumor suppressor gene. It may occur sporadically or may be family related; there are various patterns of tumor dissemination that can vary the visual and eye prognosis. The most common sign is leukocoria followed by strabismus, with an average age of onset at 2 years in unilateral cases and during the first year of life in bilateral tumors. Early detection and appropriate treatment can prolong survival in children.

Phacoemulsification and foldable acrylic IOL implantation in children with treated retinoblastoma / Facoemulsificação e implante de lente intraocular acrílica dobrável em crianças com retinoblastoma

PURPOSE: To study the results of cataract surgery in children with radiation-induced cataract after treatment for retinoblastoma. METHODS: Retrospective interventional case series. Six consecutive patients diagnosed with secondary cataracts due to radiation therapy for retinoblastoma. Intervention: Phacoemulsification and foldable acrylic intraocular lens implantation. Outcomes measu- red: Visual acuity, binocular indirect ophthalmoscopy and slit-lamp biomicroscopy. Aspirated lens material and aqueous humor samples were collected during surgery. RESULTS: Six uniocular children between 3 to 5 years of age at time of surgery were studied. The mean time interval between radiotherapy and cataract diagnosis was 22.3 months. The mean follow-up after surgery was 17.2 months (range: 12 to 23 months). All eyes achieved a clear visual axis after surgery allowing monitoring of the tumor status. None developed recurrence or retinoblastoma dissemination. Histopathological analysis of the aspired material showed no tumoral cells in all samples. All patients improved vision after cataract surgery. CONCLUSIONS: Phacoemulsification with acrylic intraocular lens implantation seems to be a safe, feasible, and effective method for the removal of radiation-induced cataracts in patients with treated retinoblastoma.

OBJETIVOS: Estudar os resultados da cirurgia da catarata induzida pela radioterapia para o tratamento do retinoblastoma em crianças. MÉTODOS: Estudo retrospectivo intervencional em série de casos onde seis pacientes consecutivos apresentaram catarata secundária à terapia por radiação para o retinoblastoma. Intervenção: Facoemulsificação e implante de lente intraocular acrílica dobrável. Foram avaliadas: acuidade visual, oftalmoscopia binocular indireta e biomicroscopia. Material para análise histológica do cristalino e do humor aquoso foi coletado durante as cirurgias. RESULTADOS: Seis crianças, entre 3 e 5 anos de idade, com catarata secundária à radiação para tratamento de retinoblastoma foram submetidas à cirurgia de facoemulsificação com implante de lente intraocular. A média do intervalo de tempo decorrido entre a radioterapia e o diagnóstico da catarata foi 22,3 meses. O período médio de seguimento após a cirurgia foi de 17,2 meses (intervalo: 12 a 23 meses). Todos os olhos melhoraram a visão e mantiveram eixo visual livre permitindo a fundoscopia para monitorar o tumor. Nenhum paciente evoluiu com recorrência ou disseminação do retinoblastoma. A análise histopatológica do material colhido resultou em ausência de células tumorais nas amostras. CONCLUSÕES: A cirurgia de facoemulsificação com implante de lente intraocular acrílica dobrável mostrou ser um procedimento seguro e efetivo para o tratamento da catarata induzida pela radioterapia em pacientes portadores de retinoblastoma.

Consideraciones clínicas, diagnósticas y de tratamiento en retinoblastoma / Clinical, diagnostic and therapeutic considerations in retinoblastoma

El retinoblastoma es el tumor intraocular primari o más frecuente en l a infancia. Su detección temprana y el inici o del tratamiento adecuado permi te mejorar dramáticamente l a sobrevida en estos niños. En este artícul o se hace una revisión general de l a enfermedad. Se empleó PubMed y se revisaron artículos representativos del tema, que permi tieran dar una idea general de los di ferentes avances alcanzados. Dada su cl ínica característica, el médico de atención primaria, es pieza fundamental en l a captación inicial del paciente. [Vi l lami l JF , Quintero LM, Serrano RA, Moreno IA. Consideraciones cl ínicas, diagnósticas y de tratamiento en retinoblastoma. MedUNAB 201 1; 14:180-187].

Retinoblastoma is the most common primary intraocular tumor in childhood. Its early detection and initiation of appropriate therapy , can dramatically improve the life expectancy in these children. This article is a general review of the disease. PubMed was employed and representative articles about the topic were selected in order to given us a general idea about the advances achieved. Due to clinical features, primary care physician is a fundamental part in the initial catchment of patient. [Villamil JF , Quintero LM, Serrano RA, Moreno IA. Clinical, diagnostic and therapeutic considerations in retinoblastoma. MedUNAB 2011; 14:180-187].

Retinoblastoma en Guatemala: diagnóstico temprano salva vidas / Retinoblastoma in Guatemala: early diagnosis saves lives

El retinoblastoma es el más común cáncer ocular de la niñez. El sitio primario es la retina y que se desarrolla en los primeros 6 años de vida. Su incidencia mundial es 1 en 18,000 niños menores de 5 años en EEUU (1) (2-4). Además, la literatura sugiere que hispanos tienen una incidencia más alta que otras razas (5-6). En EEUU se diagnostican alrededor de 300 casos al año. En Guatemala, la Unidad Nacional de Oncología Pediátrica diagnostica 27-30 casos anuales; y pone al retinoblastoma como tercer cáncer de la niñez, después de leucemias y linfomas (Datos UNOP, 2010)...

Malignant fibrous histiocytoma in a patient with history of treated retinoblastoma

Retinoblastoma is one of the most important childhood tumors with genetic implications and necessity for family counseling and long term follow up. We are presenting a 22 year-old Saudi male with history of bilateral retinoblastoma, who was initially treated by enucleation of his left eye. The histopathology showed a moderately differentiated tumor with vitreous seeding and he received chemotherapy in addition to radiotherapy to his right eye. He presented 20 years later with a right orbital tumor, frozen globe and proptosis. The excisional biopsy of his orbital mass revealed a spindle cell sarcoma with features of malignant fibrous histiocytoma. His clinical and radiological findings are presented together with the histopathologic features of his orbital tumor and brief review of secondary tumors in retinoblastoma patients

Long Term Follow-up Results of External Beam Radiotherapy as Primary Treatment for Retinoblastoma

The authors reviewed their experiences of external beam radiotherapy (EBR) as an initial treatment in retinoblastoma patients to determine its long-term effect on subsequent tumor control and complications. A total of 32 eyes in 25 patients that underwent EBR for retinoblastoma were reviewed retrospectively. The patients consisted of 21 boys and 4 girls of median age at treatment of 7.1 months. Radiation doses ranged from 35 to 59.4 Gy. The 10-yr ocular and patient survivals were 75.4% and 92.3%, respectively. Nine of the 32 eyes progressed; 7 of these were enucleated and 2 were salvaged by focal treatment. According to the Reese-Ellsworth classification, 4 of 5 eyes of Group II, 13 of 16 Group III eyes, 2 of 4 Group IV eyes, and 5 of 7 Group V eyes were retained, and of the 32 eyes, 13 had visual acuity better than 20/200. Eleven patients experienced a radiation-induced complication. No patient developed a second malignancy during follow-up. Despite the limited number of patients enrolled, EBR may provide a mean of preserving eyeball and vision for some advanced lesions.

Radiation-induced spindle cell sarcoma: A rare case report.

Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy) and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

Determination of oral microflora in irradiated ocular deformed children.

Children with a retinoblastoma usually have enucleation of eye contents and undergo radiation therapy. In this group of children a slight deformation in facial growth is exhibited on the operated side. The radiation affects the oral microflora. This study was conducted to investigate the oral microflora in this group of patients compared to normal children. Nineteen children, ages 4-15 years, were tested for oral microflora by culture. A control group was comprised of 20 normal healthy children from a primary school in Bangkok, Thailand. Paraffin-stimulated whole saliva was collected and cultured for the presence of mutans streptococci, lactobacilli and Candida species. The isolation frequencies and abundances of lactobacilli and Candida species in saliva samples of irradiated children were significantly greater than healthy children. The mutans streptococci counts were significantly different from healthy controls. Radiation therapy may have had some influence on the oral ecology of these patients, so had a higher risk of caries and candidiasis than the healthy controls. Therefore, comprehensive preventive measures should be taken in these children.

Comportamiento del retinoblastoma en el Centro Nacional de Oftalmología del 2002 al 2005 / Behavior of the retinoblastoma in the National Center of Ophthalmology of the 2002 at the 2005

Se realizo un estidio en el Centro Nacional de Oftalmología, con el objetivo de analizar el comportamiento del retinoblastoma, a la vez determinar el abordaje terapeútico, que reciben las pacientes con diagnóstico de retinoblastoma en nuestro centro. El estudio es descriptivo, transversal, su universo estuvo constituido por 22 niños que fueron ingresados al Centro Nacional de Oftalmología en el período comprendido de mayo del 2002 a mayo del 2005, se hizo un estudio de tres años. La muestra fue igual al universo siendo de 22 expedientes de niños diagnosticados de retinoblastoma y que fueron ingresados en el período en estudio. Entre mayo dle 2003 y mayo 2004 se presentó más retinoblastoma, la mayoría del área rural, no hubo predilección por sexo, la edad más frecuente fue entre los 12 a 24 meses 54.4 porciento y más frecuente la forma unilateral. Del total de pacientes se confirmó el diagnóstico en 19 casos 86.4 porciento se encontró diagnóstico diferente en tres casos 13.6 porciento. El signo mas precoz fue la leucocoria con un 68 porciento y el estrabismo con 13 porciento el 63.6 porciento se presentaron en estadio V de Reese. Dentro de los estudios de imagenología fueron los más frecuentes el ultrasonido 54 porciento, en los cuales se encontraron datos de calcificación en un 86.4 porciento. La enucleación y la exanteración en la forma más frecuente de tratamiento. Orienta que deben de realizarse campañas de educación a la población sobre el primer signo clínico de presentación de Retinoblastoma (leucocoria) para lograr que lleguen a estadios mas tempranos y no se tengan que recurrir a procedimientos cruentos, así como capacitar al personal médico y de enfermería de la atención primaria para la detección precoz de los signos mas frecuentes

Management of retinoblastoma with radiation.

OBJECTIVE: To evaluate the role of radiation therapy in the management of retinoblastoma. DESIGN: Retrospective analysis. METHOD: From January 1993 to March 1994, one hundred and eleven children (150 eyes) of retinoblastoma were referred for radiotherapy. The diagnosis was based on clinical examination and ocular ultrasonogram for both the eyes. The radiation treatment policy involved 40 Gy in 20 fractions over 4 weeks delivered with sedation for children under 1 year of age, 36 Gy in 9 fractions over 3 weeks under ketamine anesthesia for 1-4 years of age and for >4 years of age, a dose of 50 Gy in 25 fractions over 5 weeks. The initial tumor regression was evaluated by A and B mode ultrasonography and/or CT scan. RESULTS: The age distribution ranged from two months to six years (median - 20 months). Bilaterality was observed in 39 out of 111 cases (35%). The male to female ratio was 1.8:1. Eighty two of the 111 children were treated by definitive external beam radiation to one or both eyes. Fifteen cases received adjuvant radiotherapy after enucleation, and 14 had extensive disease for which palliative radiotherapy was offered. We observed a complete response in 54% of cases, partial response in 32%, and none in 14% of cases. Forty per cent (40%) eye survival was documented at the end of 28 months. The complication rate encountered was about 15%. CONCLUSION: Radiotherapy is an effective modality of treatment in significant number of patients with retinoblastoma. However, it requires appropriate fractionation, precise colimation and careful immobilization with general anesthesia.

Radiation treatment of retinoblastoma: experience in the past two decades.

This series of 193 patients represents the results in management of retinoblastoma. In cases treated 15 years ago when definitive radiotherapy and fat soluble chemotherapy were not utilized, the results of the treatment were very disappointing. The outcome was more promising after October 1985. Even if all the patients were in the advanced stages, we had a promising outcome, so effective modality of treatment is the most important prognostic factor. The other factors that influence the prognosis include the time for initial treatment, the time for starting the postoperative radiation and the efficiency of the radiation treatment. The follow-up period of 2 years after treatment is adequate to predict the outcome.

Tumores intra-oculares / Intraocular tumours

O autor apresenta a sua casuística de tumores oculares demonstrando a possibilidade do tratamento conservador nos casos específicos. Em 13 pacientes foram usadas placas radioativas contendo cobalto e suturadas à esclera. O autor discute os resultados

Situaçäo do retinoblastoma nos últimos 17 anos em Curitiba / Situation of retiblastoma on last 17 years in Curitiba

Realisou-se uma revisäo de retinoblastoma em 4 hospitais de Curitiba. Encontrou-se 92 casos nos últimos 17 anos, onde analisou-se: idade em se estabeleceu o diagnóstico, sexo, tempo decorrido entre o início dos sintomas e o seu diagnósticos, sintomas mais comuns, lateralidade e extensäo e óbito destes pacientes. Conclui-se que o tempo decorrido entre o diagnóstico e o início dos sintomas é elevado, pela falta de esclarecimento da populaçäo e falta de trabalho direcionado à classe médica. Isto deverá ser melhorado com a formaçäo de equipes multidisciplinares envolvendo oftalmologistas, pediatras, oncologistas, radioterapeutas, geneticistas, psicólogos, sanitaristas e membros da comunidade

Duplo padräo de regressäo no retinoblastoma / Double regression pattern in retinoblastoma

O retinoblastoma é suscetível de apresentar regressäo espontânea ou pós-terapia espontânea ocorre em 1-2% dos casos, sendo unilateral e unifocal geralmente. A regressäo após o uso de radioterapia classifica-se em quatro tipos diferentes conforme o aspecto morfológico. Apresentamos um paciente acompanhado no Serviço de Oftalmologia do Hospital de Clínicas de Porto Alegre, com 3 anos de idade, no qual foi diagnosticado retinoblastoma no olho esquerdo, aos quatro meses de idade, sendo enucleado neste momento. Juntamente, observou-se no olho contralateral duas massas tumorais, caracterizando o estagio Ia, de acordo com a classificaçäo de Howarth. Após dois ciclos de radioterapia e dois protocolos de quimioterapia realizados intercaladamente, no período de três anos, esses focos tumorais apresentaram sinais claros de regressäo diferentes entre si: massa tumoral periférica com padräo tipo I e outra, paramacular, com padräo tipo II de regressäo. Salientamos a coexistência de dois tipos distintos de regressäo tumoral pós-terapia no mesmo olho, fato raramente descrito na literatura pesquisada

Retinoblastoma: tratamento conservador versus estádio da lesäo / Retinoblastoma: globe saving treatment versus classification

O trabalho apresenta os resultados obtidos no tratamento de 44 olhos portadores de retinoblastoma, com a conservaçäo do globo ocular. Utilizou-se a radioterapia, a crioterapia e a fotocoagulaçäo pela luz de xenônio. A eficiência destes métodos foi tanto melhor quanto menos avançado era o estádio da doença. A tentativa de tratamento conservador quando mal sucedida pode piorar o prognóstico de vida do paciente. O uso da radioterapia pode induzir tumores secundários em portadores de retinoblastoma

O que mudou no estadiamento do retinoblastoma nas duas últimas décadas

With future identification of effective chemotherapy new studies may be focused upon patients with localized disease to reduce radiation dosage or the need for immediate resection of all involved eys. In metastic retinoblastoma, chemotherapy seems ethical, however the toxicity and response rates can be monitored. Collaborative studies multicentre rials may be necessary. There are different classification to retinoblastoma: Reese Ellsworth, CCSG childrem Cancer Study Group, St. Jude's Research Hospital and others. to the Pediatric Depatment in A. C. Camargo, Säo Paulo, Brazil, are referred patients which more than a half percent are extra ocular tumors and 80% of uni or bilateral tumors are in group V at the time of diagnosis. Since December, 1987, we have been in a Cooperative Group to Treatment of retinoblastoma in Brazil that lists in table 1 and 2 classification most widely used to retinoblastoma intra and extra ocular respectively