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ACTUALIZAN DEFINICION Y TRATAMIENTO DEL LINFOMA PRIMARIO DEL SISTEMA NERVIOSO CENTRAL

ACTUALIZAN DEFINICION Y TRATAMIENTO DEL LINFOMA PRIMARIO DEL SISTEMA NERVIOSO CENTRAL

(especial para SIIC © Derechos reservados)
El linfoma primario del sistema nervioso central puede ser considerado como un tumor cerebral curable mediante tratamiento intensivo multimodal, aunque es posible la aparición de toxicidad a largo plazo como consecuencia. El desafío actual consiste en la identificación del tratamiento combinado óptimo, que permita la curación de los pacientes que presentan esta enfermedad, sin causarles perjuicios.
poortmans9.jpg Autor:
Philip M. P. Poortmans
Columnista Experto de SIIC
Artículos publicados por Philip M. P. Poortmans
Recepción del artículo
25 de Septiembre, 2006
Aprobación
21 de Diciembre, 2006
Primera edición
13 de Septiembre, 2007
Segunda edición, ampliada y corregida
7 de Junio, 2021

Resumen
El linfoma primario del sistema nervioso central (LPSNC) es un tumor cerebral relativamente infrecuente, que representa aproximadamente el 1% de todas las neoplasias intracraneales y entre el 1% y el 2% de todos los linfomas no Hodgkin. Su incidencia es mayor en los pacientes con síndromes de inmunodeficiencia, incluido el sida. Sin embargo, el notable incremento de la incidencia de LPSNC observado durante las últimas décadas, especialmente en EE.UU., no puede ser explicado exclusivamente por el aumento de frecuencia de los síndromes de inmunodeficiencia. De acuerdo con su definición, por tratarse de un tumor cerebral primario el LPSNC no presenta metástasis fuera del sistema nervioso central. El pronóstico de los pacientes con este diagnóstico que reciben tratamiento convencional, similar al administrado para otros linfomas no Hodgkin, es muy desalentador, ya que la tasa de supervivencia es inferior al 10% a los 5 años. Numerosos estudios retrospectivos y otros prospectivos de fase II, demostraron mejoría de la supervivencia promedio, con una extensión próxima a los 3 años, al administrar quimioterapia en altas dosis, especialmente sobre la base de metotrexato. Los principales factores pronósticos desfavorables en relación con la supervivencia son la edad avanzada y la puntuación insatisfactoria en escalas de valoración del estado general o neurológico. La toxicidad a largo plazo representa una posible amenaza para los enfermos que han logrado la curación después del tratamiento multidisciplinario intensivo. Además, es necesario identificar correctamente algunos subtipos infrecuentes, pero distintivos, de LPSNC.

Palabras clave
linfoma primario del SNC, radioterapia, quimioterapia, metotrexato en altas dosis, toxicidad tardía


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Abstract
Primary central nervous system lymphoma (PCNSL) is a relatively rare primary brain tumour, accounting for about 1% of all intracranial neoplasms and for about 1% to 2% of all non-Hodgkin's lymphomas. A higher incidence is found in patients with immunodeficiency syndrome, including AIDS. The marked increase, especially in the United States, in the incidence of PCNSL over the last decades can however not entirely be explained by the increased incidence of immunodeficiency syndromes. PCNSL is, as a primary brain tumour, by definition not accompanied by localisations outside of the central nervous system. The prognosis of patients with PCNSL with conventional treatment like for other NHL is very poor, with a 5-year survival rate usually at less than 10%. With high dose chemotherapy, especially methotrexate-based, several retrospective and phase II prospective studies have showed an improvement of median survival to around 3 years. The major unfavourable prognostic factors for survival are elevated age and a bad general or neurological performance score. Late toxicity emerges as a possible treat for the patients who are cured after aggressive multimodality treatment. Some rare but distinctive subtypes of PCNSL have to be recognised.

Key words
primary CNS lymphoma, radiotherapy, chemotherapy, high-dose methotrexate, late toxicity


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Clasificación en siicsalud
Artículos originales > Expertos del Mundo >
página   www.siicsalud.com/des/expertocompleto.php/

Especialidades
Principal: Hematología, Neurología
Relacionadas: Anatomía Patológica, Farmacología, Medicina Interna



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Enviar correspondencia a:
Philip M. P. Poortmans, Dr. Bernard Verbeeten Instituut, 5000, PO Box 90120, Tilburg, Países Bajos
Patrocinio y reconocimiento:
Agradecimiento: A los colegas del EORTC Lymphoma Group y del IPCG, por la excelente colaboración en la preparación y la realización de la investigación acerca del linfoma primario del sistema nervioso central. Especialmente a Hanneke C. Kluin-Nelemans, Hanny Haaxma-Reiche, Martin Van den Bent, Gustaaf van Imhoff y Helena Harder por su activa contribución.
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