RESUMEN
En México, los tumores del sistema nervioso central representan el tercer lugar de todas las neoplasias malignas. El meduloblastoma constituye 20% de los tumores primarios del sistema nervioso central y 40% de los que se originan en el cerebelo; es el tumor maligno más frecuente en la niñez. Su origen se sitúa en la capa granular externa, normalmente migra del vermis hacia la superficie de los hemisferios cerebelosos y de ahí hacia las porciones profundas para poblar la capa granular interna de las folias. Estos tumores infiltran difusamente a través de las capas moleculares de la corteza cerebelosa por debajo de la pía, similar a lo que ocurre normalmente en las etapas embrionarias. Se diseminan por la circulación del líquido cefalorraquídeo con siembras a lo largo del espacio subaracnoideo y alrededor de la médula espinal para eventualmente salirse del sistema nervioso central y diseminarse a hueso, hígado, médula ósea y otros sitios menos frecuentes. Existen en la actualidad factores pronósticos bien definidos, así como el concepto de tratamiento multidisciplinario que ha condicionado mejores expectativas de supervivencia. El objetivo de esta revisión es actualizar el conocimiento de este tipo de tumores en nuestro país, así como los resultados terapéuticos.
In Mexico, Central Nervous System (CNS) tumors are the third most common childhood cancers. Medulloblastoma constitutes 20% of the primary CNS tumors and 40% of all cerebellar tumors, the single most common brain tumor among children. It originates over the external granular layer that normally migrates from the vermis to the surface of the cerebellum hemispheres and from there to the deep portions of the internal granular layer. These tumors infiltrate profusely the cerebellar cortex. The dissemination process can occur through the spinal fluid with seeding along the subarachnoidal space and around the spinal chord They eventually produce metastasis mainly to bone, liver, and bone marrow. There is a group of well identified prognostic factors that are relevant for each individual patient and that can be applied for multidisciplinary treatment purposes. The objective of the present review is to emphasize on new research findings and the overall survival that can be achieved with modern treatment programs.
Asunto(s)
Humanos , Preescolar , Niño , Lactante , Meduloblastoma/terapia , Neoplasias Cerebelosas/terapia , PronósticoRESUMEN
In Mexico, Central Nervous System (CNS) tumors are the third most common childhood cancers. Medulloblastoma constitutes 20% of the primary CNS tumors and 40% of all cerebellar tumors, the single most common brain tumor among children. It originates over the external granular layer that normally migrates from the vermis to the surface of the cerebellum hemispheres and from there to the deep portions of the internal granular layer. These tumors infiltrate profusely the cerebellar cortex. The dissemination process can occur through the spinal fluid with seeding along the subarachnoidal space and around the spinal chord They eventually produce metastasis mainly to bone, liver, and bone marrow. There is a group of well identified prognostic factors that are relevant for each individual patient and that can be applied for multidisciplinary treatment purposes. The objective of the present review is to emphasize on new research findings and the overall survival that can be achieved with modern treatment programs.
Asunto(s)
Neoplasias Cerebelosas/terapia , Meduloblastoma/terapia , Niño , Preescolar , Humanos , Lactante , PronósticoRESUMEN
From March 1980 to December 1995, at the Department of Neurosurgery of the Instituto Nacional de Pediatría (INP), 23 patients were treated for orbital tumors. In this communication, the authors report on the clinical presentation, age, radiological findings, visual-evoked potentials, type of surgery, histological diagnosis, and results. Two kinds of surgical techniques were employed: craniotomy with orbital roof removal (NAFZIGER) and fronto-orbitotomy. Eleven patients had optic nerve gliomas, 3 had fibrous osseous dysplasia, 2 meningiomas, neurofibromas, Langerhans cells histiocytosis, and 1 case of Anaplastic astrocytoma, Teratoma, primitive Neuroectodermal tumor, germinoma with areas of choriocarcinoma. The results were evaluated for the quality of life in regard to the Karnofsky scale, tumor recurrence and postoperative visual-evoked potentials. It is important to note the very variable histological kinds of lesions found in this series that can be approached surgically with cranio orbitotomy with good results.