Response Evaluation of Choroidal Melanoma After Brachytherapy Using Diffusion-Weighted Magnetic Resonance Imaging (DW-MRI): Preliminary Findings.

Purpose: To evaluate the role of diffusion-weighted magnetic resonance imaging (DW-MRI) in the assessment of therapeutic response in patients with choroidal melanoma treated with brachytherapy. Materials and Methods: We performed a prospective, unicentric study which included patients with choroidal melanoma and indication for brachytherapy. Three DW-MRI examinations were proposed for each patient, one before and two after treatment. The apparent diffusion coefficient (ADC) value was calculated on DW-MRI and compared with local tumor control assessed by ophthalmologic follow-up. Results: From 07/2018 to 06/2019, 19 patients were recruited, 13 of whom underwent follow-up examinations. Patients' ages ranged from 24 to 78 years and 52.9% were male. At the ocular ultrasound, the mean tumor thickness and diameter were 6.3 and 11.5 mm, respectively. Two patients (15.4%) showed signs of tumor progression during follow-up (7 and 9 months after treatment). There was no statistically significant difference in tumor size between MR before and after treatment, however, there was a significant reduction in mean ADC in patients with progression (p = 0.02). Conclusion: DW-MRI is a promising method for monitoring patients with choroidal melanoma; reduction in the mean ADC values between pre-treatment MRI and the first post-treatment MRI may be related to the lack of response to brachytherapy and increased risk of disease progression.

[The Pediatrician's ability to recognize the presenting signs and symptoms of retinoblastoma]. / O papel do pediatra no diagnóstico precoce do retinoblastoma.

BACKGROUND: Retinoblastoma is the most frequent primary intraocular tumor in children. The most common presenting sign is leukocoria and the second one is strabism. In the developing countries extra ocular disease is seen more frequently because of delayed referral. METHODS: Two hundred and thirty-nine patients with retinoblastoma admitted at the Pediatrics and Ophthalmology Departments of the Hospital do Cancer AC Camargo from 1986 to 1995 were evaluated regarding lag-time and tumor extension. RESULTS: Patients referred within less than 6 months presented a higher rate of intraocular disease compared with patients having a lag-time greater than 6 months (75% vs. 25%, p<0.001). No statistically significant difference was noted for patients with extra-ocular tumors. CONCLUSION: Early diagnosis is the gold standard for patients with Retinoblastoma and the pediatrician's ability to recognize the presenting signs and symptoms can lead to early diagnosis and efficient treatment.

Unusual primary ocular neoplasm in a child: leiomyosarcoma of the ciliary body.

Primary uveal-tract neoplasms are extremely rare in childhood; the most common lesions found are melanocytic. We report here the case of a 7-year-old girl who underwent enucleation of the right eye with clinical suspicion of choroid melanoma as a result of a ciliary body mass that extended to the posterior chamber. Histologically, the neoplasm featured spindle cell morphology, atypia, and mitoses. The tumor expressed smooth muscle alpha actin, pan-actin HHF-35, and desmin, whereas immunohistochemistry for melanocytic markers, such as S-100, Melan-A, and HMB-45, was negative. Based on these features, the diagnosis of leiomyosarcoma of the ciliary body was firmly established. Although several leiomyomas have been reported in the literature, there are only 2 previously reported cases of primary leiomyosarcoma of the uveal tract. Immunohistochemical expression of muscle proteins allowed distinction from the most common melanocytic tumors arising in this location.

Retinoblastoma in children older than 5 years of age.

BACKGROUND: Retinoblastoma is a malignant tumor of the embryonic neural retina. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years. OBJECTIVE: To determine characteristics and prognosis of retinoblastoma in children older than 5 years. PROCEDURES: From 1986 to 2002, medical records of 16 patients out of 453 cases referred to Hospital do Câncer AC Camargo, São Paulo, Brazil. RESULTS: Median age at diagnosis was 73.7 months (range 65-144) and there was an equal gender distribution. Fifteen patients presented with unilateral disease. The mean time between first symptoms and diagnosis was 9.6 months (range 0-48). Most cases were diagnosed in advanced stages and 15 eyes were enucleated. Eleven patients presented with intraocular tumor (1 Reese II and 10 Reese V) and five presented with extraocular disease (one CCG II and four CCG III). Twelve patients are still alive with a median follow-up of 92 months (range 65-199). CONCLUSIONS: Because of its low incidence at this age, diagnosis of retinoblastoma is usually delayed due to low level of suspicion. Therefore, it is important that physicians are aware of this disease in order to perform an earlier diagnosis, and decrease treatment-related morbidity.

Trilateral retinoblastoma.

BACKGROUND: Trilateral retinoblastoma (TRB) is a syndrome consisting of unilateral or bilateral hereditary retinoblastoma (Rb) associated with an intracranial neuroblastic tumor. Although its incidence is low, the prognosis is very poor. This article reports four cases of TRB and discusses the role of neuroimaging screening for early detection. PROCEDURE: From January 1986 to December 2003, 470 children with Rb were admitted to the Pediatrics and Ophthalmology Departments, A C Camargo Hospital, São Paulo, Brazil. RESULTS: There were four patients with pineoblastoma, two of whom had a positive familial history. The age at diagnosis of Rb was 4, 6, 10, and 24 months while the age of diagnosis of TRB was 10, 25, 57, and 72 months. One patient presented TRB at initial diagnosis of Rb. Three patients had bilateral disease and all of them had one eye enucleated, followed by chemotherapy and/or external beam radiation therapy (EBRT). One child with unilateral disease was only submitted to enucleation. In spite of intensive treatment, all patients died with progressive disease within 7, 8, 12, and 12 months after diagnosis of TRB. CONCLUSIONS: Early diagnosis as well as new therapeutic approaches are needed to achieve better results.

The addition of ifosfamide/etoposide to cisplatin/teniposide improves the survival of children with retinoblastoma and orbital involvement.

This study aimed to determine the impact of the addition of ifosfamide/etoposide to a regimen containing cisplatin/teniposide on the survival of patients with retinoblastoma with orbital involvement. Thirty patients were treated at the A. C. Camargo Hospital, Brazil, from 1986 to 2002. From 1986 to April 1992 (period I, n=12), treatment consisted of 3 cycles of induction chemotherapy with cisplatin and teniposide, followed by maintenance with same drugs alternating with cyclophosphamide, vincristine, and doxorubicin every 21 days for 60 weeks. Since April 1992 (period II, n=18), the treatment consisted of 3 cycles of ifosfamide and etoposide followed by maintenance with same drugs, alternating with cisplatin and teniposide every 21 days for 36 weeks. In both periods, children were submitted to exenteration with eyelid preservation and orbital radiation therapy with 45 cGy, and also received intrathecal therapy with methotrexate plus dexamethasone and cytarabine. Kaplan-Meier method was used for survival analysis. The median age was 31 months. Most patients (86.7%) presented unilateral tumors. The 3-year overall survival was 34.4% and 72.2%, respectively, for patients treated during periods I and II (P=0.061). The addition of ifosfamide/etoposide to chemotherapy with cisplatin/teniposide improves survival in these patients, but further studies are still necessary.

Treatment of retinoblastoma patients with chemoreduction plus local therapy: experience of the AC Camargo Hospital, Brazil.

To evaluate the efficacy of conservative management of intraocular retinoblastoma with chemoreduction combined with local therapy with or without plaque radiation in the preservation of the eye, and avoidance of external beam radiation therapy (EBRT) (success rate). From 1995 to 2000, 84 newly diagnosed patients with intraocular retinoblastoma were admitted to the Pediatric Department of the Hospital do Cancer A.C. Camargo, São Paulo, Brazil. All children were treated with 2 to 6 cycles of chemotherapy (carboplatin, vincristine, and etoposide) plus local therapy (cryotherapy, laser photocoagulation, and thermotherapy), or plaque radiation therapy during and/or after the chemotherapy. The Mann-Whitney test was used to compare means of quantitative variables. The chi test or the Fisher exact test were employed to verify the association between the outcome and the independent variables. For all tests alpha=5% was adopted. Success rate was higher for patients with bilateral tumors (54%) than for children with unilateral tumors (19%) (P=0.003). For patients with Reese-Ellsworth stages I, II, and III, no statistically significant differences in the success rates were noted in the group of unilateral (50%) and bilateral tumors (79.1%) (P=0.179). Among children with Reese-Ellsworth stages IV and V, the success rate was significantly higher for patients with bilateral tumors (40.7%) than for those with unilateral (0%) (P=0.012). Chemoreduction combined with local therapy, with or without plaque radiotherapy, is efficacious in avoiding enucleation and the use of external beam radiation therapy for children with intraocular retinoblastoma.

Extraocular retinoblastoma: a 13-year experience.

BACKGROUND: The current study was performed to evaluate two regimens of treatment and to describe clinical and epidemiologic characteristics in patients with extraocular retinoblastoma. METHODS: Eighty-three patients with extraocular retinoblastoma according to Childrens Cancer Group (CCG) classification were admitted to the Pediatric Department of the A. C. Camargo between 1987-2000. The age, gender, race, lag time, first clinical presentation, staging, laterality, and treatment regimen were analyzed. Treatment was comprised of cisplatin, teniposide, vincristine, doxorubicin, and cyclophosphamide during the first treatment period (1987-1991) or cisplatin and teniposide with alternating courses of ifosfamide and etoposide during the second treatment period (1992-2000). RESULTS: The mean age of the patients was 32.9 months (range, 2-145 months). The mean lag time was 10.5 months. Forty-three patients were treated in the first period and 40 patients were treated in the second period. Locally advanced tumors (Class I-III) were present in 83.1% of the patients. There was a positive correlation between lag time and age for unilateral tumors (correlation coefficient [r] = 0.35; P = 0.006), whereas the correlation was negative for bilateral tumors (r = -0.12; P = 0.63). The 5-year overall survival was 55.1% in the first treatment period and 59.4% in the second treatment period (P = 0.69). No significant differences with regard to survival rates were noted for unilateral tumors between the two treatment periods (44.6 noted for unilateral tumors vs. 59.1 noted for unilateral tumors). CONCLUSIONS: In the current study, the addition of ifosfamide and etoposide to a treatment regimen comprised of cisplatin, teniposide, vincristine, doxorubicin, and cyclophosphamide did not appear to improve the survival of patients with extraocular retinoblastoma. Patients with dissemination to the central nervous system or metastatic disease remain incurable and die of progressive disease, despite the aggressive treatment. A multicenter trial should be considered to evaluate the best strategy for these situations.

Termoterapia transpupilar em melanoma maligno da coróide / Transpupillary thermotherapy in choroidal malignant melanoma

Objetivo: Vários métodos vem sendo utilizados para o tratamento dos melanomas da coróide. A proposta deste trabalho preliminar é avaliar a eficácia da termoterapia transpupilar (TTT) como tratamento primário de melanomas da coróide pequenos. Métodos: Foi realizado um trabalho prospectivo e näo-randomizado para avaliar os aspectos clínicos, resposta do tumor, complicaçöes e resultados visuais de pacientes portadores de melanomas da coróide pequenos (até 4,0 mm de espessura e 12 mm de diâmetro basal) tratados por termoterapia transpupilar utilizando-se sucessivas aplicaçöes de laser diodo continuo de 810 nm. Resultados: Foram tratados 11 pacientes portadores de melanomas da coróide pequenos. O tumor era único e pigmentado em 100 por cento dos casos. Crescimento documentado esteve presente em 5 pacientes (45,45 por cento) previamente ao tratamento e fatores de risco para crescimento ou metástase estavam presentes em todos os pacientes. O tempo de seguimento destes pacientes a partir do tratamento foi em média de 5,72 meses (3 -8 meses ). Foram utilizadas 3 sessöes de laser em 5 pacientes 45,45 por cento) e 4 sessöes em 6 pacientes (64,64 por cento). As lesöes apresentavam, por ocasiäo do diagnóstico, uma espessura média de 2,65 mm (1,85-3,86 mm), com maior diâmetro basal médio de 7,98 mm(4,2-11,33 mm). Após o tratamento, a espessura média foi de 1,83 mm (0,98-2,93mm) e o maior diâmetro basal médio foi de 6,59mm (3,81 mm -10,67 mm). Das lesöes tratadas, l 00 por cento apresentaram diminuiçäo da altura e do máximo diâmetro basal, tendo sido a diminuiçäo média da espessura de 0,89 mm e do máximo diâmetro basal de 1,39 mm. A acuidade visual manteve-se inalterada em 5 casos (45,45 por cento) e piorou após o tratamento em 6 casos (54,54 por cento). Ocorreram complicaçöes em 9 casos, tendo sido considerada complicaçäo grave I caso de descolamento parcial da retina (9,09 por cento); as outras complicaçöes foram consideradas leves: pequenas hemorragias intra-retinianas em 7 pacientes (63,63 por cento), vitreite associada a tênues membranas vitreas em 1 paciente (9,09 por cento) e quemose associada a edema palpebral em I paciente (9,09 por cento). Controle tumoral local com conservaçöo do globo ocular foi observado durante este pequeno tempo de seguimento em 100 por cento dos pacientes tratados. Por ocasiäo da "última revisäo", 100 por cento dos pacientes estavam vivos e sem doença metastática...

O papel do pediatra no diagnóstico precoce do retinoblastoma / The Pediatrician's ability to recognize the presenting signs and symptoms of retinoblastoma

OBJETIVO: O retinoblastoma é o tumor intra-ocular mais freqüente na infância. A forma de apresentação mais comum é leucocoria, seguida pelo estrabismo. Nos países em desenvolvimento, pela demora diagnóstica e maior tempo de encaminhamento, encontramos um grande número de pacientes portadores de tumores extra-oculares. MÉTODOS: Duzentos e trinta e nove pacientes com retinoblastoma admitidos nos departamentos de Pediatria e Oftalmologia do Hospital do Câncer AC Camargo, no período de 1986 a 1995, foram avaliados quanto ao tempo de encaminhamento e extensão do tumor. A análise estatística incluiu o cálculo de freqüências absolutas e relativas, bem como o teste exato de Fisher, para a verificação da associação entre variáveis categorias. RESULTADOS: Pacientes que foram encaminhados em um período menor que 6 meses apresentaram maior freqüência de doença intra-ocular quando comparados com pacientes com tempo de encaminhamento maior que seis meses (75 por cento vs. 25 por cento p<0,001). Não houve diferença estatisticamente significativa para tumores extra-oculares. CONCLUSÕES: O diagnóstico precoce é considerado o ponto mais importante no tratamento de pacientes com retinoblastoma. Os pediatras são capazes de detectar, em uma consulta simples de ambulatório, os sinais e sintomas desta doença e devem ter sempre em mente o diagnóstico de retinoblastoma, possibilitando o encaminhamento precoce a centros especializados.

Achados ultrassonográficos em pacientes com melanoma uveal submetidos à braquiterapia / Ultrassonographic findings in patients with uveal melanoma submited to brachytherapy

Cinquenta e oito pacientes com diagnóstico clínico de melanoma da coróide, submetidos a tratamento conservador no período de dezembro de 1988 a 1994, foram avaliados por ultrassonografia pré e pós tratamento. Os aspectos analisados ao diagnóstico e suas alteraçöes durante o período de seguimento foram: a refletividade interna ao diagnóstico foi média/baixa com "ângulo Kappa" pronunciado em 86,2 por cento dos casos, média em 9,8 por cento e baixa em 3,5 por cento, tendo ocorrido variaçäo evidente no padräo de refletividade em 39,2 por cento dos tumores após o tratamento. Com relaçäo às dimensöes dos tumores, houve: reduçäo na altura dos mesmos em 64 por cento dos pacientes, inalteraçäo em 28 por cento e aumento em 8 por cento dos casos. Reduçäo concomitante da altura e base dos tumores ocorreu em 12 pacientes (24 por cento). Escavaçäo da coróide estava presente em 90,5 por cento dos casos, näo tendo sido observada variaçäo neste parâmetro após o tratamento. Ruptura da membrana de Bruch foi encontrada em 41,3 por cento dos tumores ao diagnóstico, tendo ocorrido em 3 casos após o tratamento. Descolamento da retina foi verificado em 72 por cento dos pacientes ao diagnóstico, com desaparecimento do mesmo em 38,8 por cento dos pacientes, aumento em 5,5 por cento e em 38,8 por cento dos casos com o D.R. näo se alterou durante o seguimento. Hemorragia vítrea ocorreu em 5 pacientes (8,6 por cento) após a terapia. Edema escleral e/ou do espaço de Tenon foi evidenciado em 76,3 por cento dos casos após braquiterapia. Infiltraçäo escleral pelo tumor foi sugerida pelo U.S. em 03 casos dos quais 2 com comprovaçäo anátomo-patológica. O U.S. falhou na demonstraçäo de um caso de invasäo escleral detectada ao A.P

Ultrasound biomicroscopy and secondary epithelial downgrowth cystis of anterior chamber

Objetivos: Determinaçäo de correlaçäo clínica e ultra sonográfica de lesöes císticas de implantaçäo secundária em íris e câmara anterior, referidas após trauma ou cirurgia intra-ocular. Material e Métodos: Estudo retrospectivo de exames de biomicroscopia ultra-sônica (UBM Modelo 840 - Humphrey Instruments, EUA, equipado com transdutor de 50 MHz utilizando-se a técnica de imersäo), atendidos de agosto de 1994 a julho de 1997 no setor de Ultra-Sonografia Ocular da Unifesp-EPM. Resultados: Dentre 1669 exames de UBM, foram encontrados 8 casos de cistos epiteliais de implantaçäo secundária, sendo 3 após trauma (2 sob forma de lesäo amarelo-esbranquiçada que ao exame anátomo-patológico demontrou conteúdo de lamelas concêntricas de queratina; outro com suspeita de trauma penetrante auto-selante com lesäo de conteúdo hipodenso) e 5 após cirurgia intra-ocular (pós-facectomia isolada, 3 casos; pós transplante penetrante de córnea, 2 casos). Um dos casos pós-facectomia extracapsular referia trauma penetrante pregresso. A UBM, identificaram-se os cistos de implantaçäo secundária como lesöes unilaterais, únicas, de conteúdo hipodenso (com partículas em suspensäo) ou dispostas em camadas hiper-refletivas(lamelas de queratina, no caso dos cistos de aspecto perláceos, cuja origem está nas células de folículo piloso implantado). Em todos os casos de conteúdo hipodenso (6) observou-se a delaminaçäo do estroma iriano, informaçäo näo disponível por meio de outros métodos. Pelo do exame de UBM realizou-se o acompanhamento pós-operatório em 2 casos (1 submetido a exérese cirúrgica, outro à cistotomia com laser) ou entäo o exame seriado se decidido pela observaçäo clínica. Conclusäo: O exame de UBM pôde diferenciar os tipos de epitelizaçäo de câmara anterior (forma cística ou plana), detectar lesöes associadas indicando tratamento mais adequado, e o acompanhamento destas lesöes após tratamento ou näo.

Leiomioma mesectodérmico de íris e corpo ciliar - relato de caso / Mesectodermal leiomyoma of the iris and ciliary body: case report

Objetivo: Descrever um caso de leiomioma mesectodérmico de íris e corpo ciliar. Local: Hospital do Câncer - A. C. Camargo - Fundação Antônio Prudente. Método: Relato de caso e revisão da literatura. Relato de caso: O paciente apresentava uma lesão de aspecto sólido, arredondada, avermelhada, ricamente vascularizada em quadrante temporal inferior de íris de olho esquerdo. À biomicroscopia ultrassônica havia extensão ao corpo ciliar. As hipóteses diagnósticas foram: tumor metastático, melanoma amelanótico e outro tumor a esclarecer. Foi realizada a enucleação do olho acometido e o resultado anatomopatológico e imuno-histoquímico revelou leiomioma mesectodérmico de íris e corpo ciliar. Conclusão: O leiomioma mesectodérmico de trato uveal é um tumor benigno e raro. As decisões de conduta podem ser difíceis em função do seu aspecto, tamanho e localização.