RESUMEN
Cerebral ganglioneuroblastoma is an embryonal tumor of the central nervous system, which has been rarely encountered into the spinal cord. The standard treatment for ganglioneuroblastoma is complete surgical excision. A 15-year old boy was presented with cord compression. Magnetic resonance imaging revealed an intradural and intramedullar enhancing lesion over T2 spine. A histomorphological diagnosis was made in the presence of immature small round cells admixed with a good number of ganglion cells. The morphological diagnosis was verified by immunohistochemistry. This is the first reported case of compressive myelopathy in the thoracic region of the spine.
Asunto(s)
Adolescente , Ganglioneuroblastoma/diagnóstico , Ganglioneuroblastoma/epidemiología , Ganglioneuroblastoma/cirugía , Humanos , Masculino , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/epidemiología , Neoplasias de la Médula Espinal/cirugía , Vértebras Torácicas/patologíaRESUMEN
In this case report we describe a case of mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide [VIP], causing secretory diarrhoea in an 18-month-old child. An 18-month-old girl presented with a 2-month history of diarrhoea, abdominal distension and weight loss. Investigations revealed secretory diarrhoea with hypokalaemia, hyponatraemia and hypochloraemia and metabolic acidosis. Her stool output was 2.5-3.1 day[-1] with increased stool sodium. VIP levels were strikingly high with normal glucagon and gastrin levels. X-ray of the chest revealed a well-defined mass in the right upper zone with tracheal shift, which was confirmed with computed tomography [CT] of the chest. The mass was resected and the patient became asymptomatic. This case shows that secretory diarrhoea caused by VIP and produced by ganglioneuroblastoma indicates a favourable prognosis, provided it is resectable
Asunto(s)
Humanos , Femenino , Neoplasias del Mediastino , Diarrea/etiología , Péptido Intestinal Vasoactivo , Ganglioneuroblastoma/cirugía , HipopotasemiaRESUMEN
Los tumores neuroblásticos son tumores del sistema nervioso periférico. Algunos inmaduros como el neuroblastoma tienen comportamiento muy agresivo, mientras otros como el ganglioneuroma son considerados benignos. Por su variada sintomatología, al neuroblastoma se le denomina "el gran simulador" y es caracterizado como enigmático. El dolor es la principal manifestación, así como también la distensión abdominal y la palpación de una masa abdominal.
Asunto(s)
Humanos , Femenino , Niño , Ganglioneuroblastoma/cirugía , Ganglioneuroblastoma/diagnóstico , Ganglioneuroblastoma/patología , Neoplasias Pancreáticas/diagnóstico , Dolor Abdominal/etiología , Estadificación de Neoplasias , Neuroblastoma/clasificación , Neuroblastoma/genética , Neuroblastoma/patologíaRESUMEN
OBJECTIVE: To report a case of ganglioneuroblastoma of cerebellum, with emphasis to the neuroimaging and pathological findings. CASE REPORT: A one year and eight-month-old girl presented with a two-month history of hypoactivity and tremor in the legs. The MRI showed an enhancing cerebellar mass hypointense on T1 and hyperintense on T2-weighted images. The patient underwent a craniotomy with resection of the lesion. The histological and immunohistochemical studies defined the diagnosis of ganglioneuroblastoma. CONCLUSION: The MRI findings of our case showed no features which could help in the differentiation between ganglioneuroblastoma and the other common types of posterior fossa neoplasms in the pediatric population.
OBJETIVO: Relatar um caso de ganglioneuroblastoma no cerebelo, com ênfase aos achados de imagem e patologia. RELATO DO CASO: Paciente feminino de um ano e oito meses apresentou-se com hipoatividade e tremor nas pernas há dois meses. A RM demonstrou uma massa cerebelar hipercaptante, com hipossinal em T1 e hipersinal em T2. A paciente foi submetida a craniotomia com ressecção da lesão. Os exames histológicos e imuno-histoquímicos definiram o diagnóstico de ganglioneuroblastoma. CONCLUSÃO: Os achados de RM deste caso não demonstraram padrões que pudessem auxiliar na diferenciação entre ganglioneuroblastoma e os demais tumores que comumente acometem a fossa posterior de crianças.
Asunto(s)
Femenino , Humanos , Lactante , Neoplasias Cerebelosas/patología , Cerebelo/patología , Ganglioneuroblastoma/patología , Craneotomía , Neoplasias Cerebelosas/cirugía , Diagnóstico Diferencial , Ganglioneuroblastoma/cirugía , Neoplasias Infratentoriales/patología , Imagen por Resonancia MagnéticaRESUMEN
En dos de lactantes mayores con síndrome de opsoclonus mioclonus (ataxia, opsoclonus y polimioclonías), la búsqueda dirigida con métodos de laboratorio e imágenes, permitió detectar sendos ganglioneuroblastomas retroperitoneales en etapas II y III de Evans, repectivamente, uno y doce meses después del inicio de los síntomas neurológicos. Ambos fueron tratados con cirugía y quimioterapia, con regresión del sindrome neurológico y evolución posterior satisfactoria. Se han comunicado alrededor de 110 casos de opsoclonus mioclonus, 50 por ciento de ellos asociados a neuroblastoma oculto. La asociación tiene un pronóstico más favorable que el de otros neuroblastomas. Cuando se identifica el síndrome debe buscarse exhaustivamente el tumor