RESUMEN
A 59-year-old woman presented with disturbance of consciousness and decreased visual acuity caused by a suprasellar mass identified on MRI. A bifrontal interhemispheric approach allowed removal of the top and lateral sides of the tumor from the wall of the third ventricle. The hypothalamus appeared to be the origin of the mass, which proved to be hemangioblastoma, a rare tumor in this location.
Asunto(s)
Neoplasias del Ventrículo Cerebral/patología , Hemangioblastoma/patología , Neoplasias Hipotalámicas/patología , Hipotálamo/patología , Tercer Ventrículo/patología , Antígenos CD34/análisis , Antígenos CD34/metabolismo , Biomarcadores de Tumor , Capilares/patología , Neoplasias del Ventrículo Cerebral/irrigación sanguínea , Neoplasias del Ventrículo Cerebral/cirugía , Trastornos de la Conciencia/etiología , Femenino , Hemangioblastoma/irrigación sanguínea , Hemangioblastoma/cirugía , Humanos , Neoplasias Hipotalámicas/irrigación sanguínea , Neoplasias Hipotalámicas/cirugía , Imagen por Resonancia Magnética , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/análisis , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Silla Turca/anatomía & histología , Células del Estroma/patología , Tercer Ventrículo/cirugía , Resultado del Tratamiento , Baja Visión/etiologíaRESUMEN
OBJECT: The authors' aim in conducting this study was to investigate retrospectively the prognostic significance of angiogenic features in optic pathway/hypothalamic gliomas (OPHGs) in children. METHODS: Patients were identified in whom a diagnosis of OPHG was made using pathological analysis at the Toronto Hospital for Sick Children between 1985 and 2002. Tumor specimens were reviewed for diagnostic accuracy and adequacy of the specimen. Sections were immunostained with factor VIII to assess microvessel density (MVD). A ratio of alpha-smooth muscle actin to factor VIII immunostaining was calculated to arrive at a vascular maturity index (VMI). Vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) immunostaining were performed to evaluate angiogenic factors. In addition, the MIB-1 labeling index (LI) was used to assess proliferation. These factors were evaluated with respect to progression-free survival (PFS). Forty-one of 60 patients originally identified had adequate samples and follow up for inclusion in the study. Of these, eight patients had coexisting neurofibromatosis Type 1. Twenty-eight patients experienced tumor progression after the initial treatment (surgery with or without adjuvant treatment). Thirty-eight patients are still alive. A high MVD (> 21 vessels/1.2 mm2) was associated with a significantly higher rate of progression compared with a low MVD (< 21 vessels/1.2 mm2; p = 0.017). Microvessel density was also predictive of reduced PFS on multivariate analysis stratified for extent of resection (p = 0.04), and VMI as well as intensity and distribution of VEGF and VEGFR staining and the MIB-1 LI were not significantly associated with PFS. CONCLUSIONS: These findings suggest that MVD is the best current predictor of PFS in incompletely resected OPHGs. This information highlights the importance of angiogenesis in regard to low-grade gliomas.
Asunto(s)
Neoplasias Hipotalámicas/irrigación sanguínea , Neovascularización Patológica/patología , Glioma del Nervio Óptico/irrigación sanguínea , Adolescente , Biomarcadores de Tumor/análisis , División Celular/fisiología , Niño , Preescolar , Progresión de la Enfermedad , Humanos , Neoplasias Hipotalámicas/patología , Neoplasias Hipotalámicas/cirugía , Técnicas para Inmunoenzimas , Lactante , Microcirculación/patología , Neoplasia Residual/irrigación sanguínea , Neoplasia Residual/patología , Neovascularización Patológica/cirugía , Glioma del Nervio Óptico/patología , Glioma del Nervio Óptico/cirugía , Pronóstico , Estudios RetrospectivosRESUMEN
We report five cases of tumors occurring in three children and in two adults. The tumors had unusual histomorphology and a mixture of ependymal and piloid-like astrocytic features and a myxoid stroma similar to myxopapillary ependymomas. MR imaging in three of the cases showed aggressive, intensely enhancing partially cystic hypothalamic-suprasellar masses near midline and near the floor of the third ventricle. In the three pediatric cases, the tumor encased the circle of Willis. This newly characterized tumor, the tanycytoma, has neoplastic cells with histomorphologic and ultrastructural characteristics similar to those of the tanycyte.