Meduloepitelioma de cuerpo ciliar. Reporte de un caso y revisión de la literatura / Ciliary body medulloepithelioma. Case report and literature review

Introducción: Se presenta el caso de un paciente de 10 años de edad a quien se le realizó el diagnóstico de meduloepitelioma de cuerpo ciliar con posterior enucleación. Objetivo: Presentar las principales características clínicas, abordaje diagnostico y terapéutico de un paciente con meduloepitelioma de cuerpo cilar, realizando una revisión de la literatura existente de esta patología. Diseño del estudio: Reporte de caso. Resumen del caso: Paciente masculino de 10 años de edad que presentó perdida de agudeza visual asociada a dolor ocular, al examen oftalmológico se encontró masa en cámara anterior. Se realizó biopsia de lesión iridiana diagnosticándose meduloepitelioma de cuerpo ciliar con posterior enucleación. Conclusión: Se presentó el caso de un niño con meduloepitelioma de cuerpo ciliar, exponiéndose las manifestaciones clínicas, su diagnóstico y tratamiento, así como la revisión de la literatura existente.

Background: We present the case of a 10-year-old patient who underwent a diagnosis of ciliary body medulloepithelioma with subsequent enucleation. Objective: To present the main clinical characteristics, diagnostic and therapeutic aproach of a patient with a ciliary body medulloepithelioma, carrying out a review of the existing literature of this pathology. Study design: Case report. Case summary: A 10-year-old male patient presented with loss of visual acuity associated with ocular pain. Ophthalmological examination revealed a mass in the anterior chamber. A biopsy of an iris lesion was made, diagnosing a ciliary body medulloepithelioma with subsequent enucleation. Conclusion: The case of a child with ciliary body medulloepithelioma was presented, exposing the clinical manifestations, their diagnosis and treatment, as well as the review of the existing literature.

Análise de sobrevida e fatores prognósticos de pacientes pediátricos com tumores cerebrais / Analysis of survival and prognostic factors of pediatric patients with brain tumor

OBJETIVOS: Realizar análise de sobrevida e avaliar, através de análise multivariada, a influência de diversas variáveis na sobrevida, definindo fatores prognósticos de pacientes pediátricos com tumores do sistema nervoso central (SNC) tratados em um único centro. MÉTODOS: Analisamos, retrospectivamente, a sobrevida de 103 crianças portadoras de tumores cerebrais primários, diagnosticadas consecutivamente no período entre janeiro de 2000 e dezembro de 2006. Análise multivariada de fatores influenciando a sobrevida global por regressão de Cox foi usada para definir possíveis fatores prognósticos. RESULTADOS: A mediana e a média de idade foram de 7,2 e 7,6 anos. Houve predominância do sexo masculino (relação 1,22:1). A maioria dos pacientes tinha meduloblastoma ou tumores neuroectodérmicos primitivos (PNET, 38 por cento) ou astrocitomas de baixo grau (18 por cento). As topografias mais comuns foram cerebelar (49 por cento) e tronco cerebral (21 por cento). A sobrevida, 5 anos após o diagnóstico, foi de 84 por cento para astrocitomas de baixo grau e 51 por cento para meduloblastomas e PNET. Fatores prognósticos para a sobrevida global foram histopatológico (astrocitomas de alto grau e ependimomas, razão de risco entre 3,7 e 3,9), cirurgia (razão de risco 0,5 para tumores completamente ressecados) e radioterapia (razão de risco 0,5 para pacientes que receberam radioterapia). CONCLUSÕES: A sobrevida global de pacientes pediátricos com tumores cerebrais neste estudo é comparável àquela dos registros populacionais dos Estados Unidos e Europa. Os fatores de prognóstico definidos para sobrevida global também se assemelham àqueles previamente publicados.

OBJECTIVES: To estimate survival and evaluate prognostic factors of pediatric patients with central nervous system (CNS) tumors treated in a single center. METHODS: Retrospective analysis of survival of 103 children with primary brain tumors diagnosed consecutively from January 2000 to December 2006. Cox regression was used for multivariate analysis of factors that affect overall survival to define possible prognostic factors. RESULTS: Median and mean ages were 7.2 and 7.6 years. There was a male predominance (1.22:1). Most patients had medulloblastomas or primitive neuroectodermal tumors (PNET, 38 percent), or low-grade astrocytomas (18 percent). The anatomic site of most tumors was the cerebellum (49 percent) and the brain stem (21 percent). Five-year survival after diagnosis was 84 percent for low-grade astrocytomas and 51 percent for medulloblastomas and PNET. Prognostic factors for overall survival were histopathological type (high-grade astrocytomas and ependymomas; hazard ratio = 3.7 to 3.9), surgery (hazard ratio of 0.5 for completely resected tumors) and radiotherapy (hazard ratio of 0.5 for patients who underwent radiotherapy). CONCLUSIONS: Overall survival of pediatric patients with brain tumors in this study was similar to that found in populations of the United States and Europe. The prognostic factors defined for overall survival are also similar to those published in previous studies.

Derrame pleural incomum: metástase pleuropulmonar de tumor neuroectodérmico primitivo / Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor

O tumor neuroectodérmico primitivo é uma neoplasia com diferenciação neural de comportamento invasivo que origina metástases para diversos órgãos. Relatamos um caso de tumor neuroectodérmico primitivo primário em axila com metástases para pulmão, pleura, osso, músculo ilíaco e medula óssea. Enfatizamos o achado incomum da análise citológica do líquido pleural.

Primitive neuroectodermal tumor is an invasive neoplasm with neuronal differentiation, which frequently results in metastasis in various organs. We report the case of a patient with primitive neuroectodermal tumor whose primary site was the axilla. The patient presented with metastases in the lung, pleura, bone, iliac muscle and bone marrow. We highlight the uncommon finding in the pleural fluid cytology.

Primtive neuroetodermal tumors in childhood Abour four cases

Peripheral primitive neuroectodermal tumors are malignant small round cell tumors which occur rarely in children we retrospectively reviwed the clinical data and radiological studies of four such cases. the youngest child had a pelvic tumor and each of two children had a chest wall tumor. the fourth patient had a bone swelling tumors were all diagnosed by histological and immunhistochimical examinations most patients had combined treatment with chemotherapy surgery and radiation therapy. Best results were obtained in two cases and another died. in this report the clinical aspects of PNET in children are described reviewing the presentation of the disease in four patients and detailing the treatment strategies

Metástase cerebral de tumor de Askin. Relato de caso / Brain metastasis from Askin's tumor. Case report

Tumores neuroectodérmicos primitivos (PNET) são tumores originados de células pluripotentes da crista neural e podem ocorrer tanto no sistema nervoso central (SNC) como fora dele. Na maioria das vezes têm comportamento biológico maligno. A variante toracopulmonar dessas lesões é denominada tumor de Askin. Metástases no SNC são pouco descritas na litertura, sendo difícil estimar sua incidência e aferir a importância terapêutica e prognóstica. Os autores relatam um caso de um paciente com metástase cerebral de um tumor de Askin, realçando aspectos de tratamento e prognóstico. Mais relatos são necessários para se verificar o impacto da metástase no SNC na sobrevida desses pacientes.

Tumor neuroectodérmico primitivo na infância: relato de 13 casos e revisão da literatura / Primitive neuroectodermic tumor in childwood: a report of 13 cases and literature review

Proposta: a proposta desse estudo é a análise retrospectiva dos pacientes com diagnóstico de tumor neuroectodérmico primitivo (PNET), admitidos no Departamento de Pediatria do Hospital do Câncer no periodo de 1989 a 1996. Pacientes e métodos: Ttdos os 13 pacientes portadores de PNET foram analisados retrospectivamente utilizando uma ficha para obtenção dos dados epidemiológicos clínicos, terapêuticos e seguimento de cada paciente. Resultados: a cirurgia foi a primeira abordagem terapêutica em 2 pacientes. Quimioterapia foi administrada em 12 pacientes e radioterapia em 2. Dos 13 pacientes 5 estão vivos em seguimento médio de 48 meses após o término do tratamento. Conclusão: a análise dos 13 pacientes com PNET e revisão da literatura demonstram a agressividade desse tumor. Devido a raridade dessa patologia, estudos multiinstitucionais, podem ter papel importante para análise de fatores prognósticos e consequente abordagem terapêutica.

Primitive peripheral neuroectodermal tumors Mayagüez Medical Center experience

Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)

Perspectives in the management of central nervous system tumours.

A general review and update in the management of CNS tumours using medulloblastoma as the main model is given in this article. Special emphasis has been placed on the benefits of combined modality treatment for brain tumours. The pathogenesis and management of these tumours is discussed and recommendations made for treatment in developing countries.