Gangliogliomas intracraneales. Revisión de una serie de 20 pacientes / Intracranial gangliogliomas. A review of a series of 20 patients

Introducción: El ganglioglioma es un tumor primario, de bajo grado, del sistema nervioso central constituido por una población celular mixta de elementos gliales y neuronales. Representan entre el 0,4 al 2% de todos los tumores intracraneales y afectan fundamentalmente a niños y adultos jóvenes. Métodos: Entre los años 1995 y 2008 hemos tratado en nuestro hospital 20 pacientes (12 adultos y 8 niños) con ganglioglioma intracraneal. Revisamos retrospectivamente el sexo, el síntoma de inicio y la edad, sintomatología y tiempo de evolución, exploración neurológica, localización del tumor, aspecto en la tomografía computarizada y resonancia magnética, el tratamiento quirúrgico y la evolución. Todos los pacientes fueron intervenidos quirúrgicamente y la extensión de la resección fue evaluada de la hoja operatoria y del seguimiento neurorradiológico. Resultados: La media de edad de los pacientes fue de 26,4 años (rango 1-75) y el ratio mujer/varón fue de 1.5:1. Excepto en un caso, todos los pacientes debutaron con crisis epilépticas, con una duración media antes del diagnóstico de 7,4 años (rango 1-29). Diecisiete tumores estaban localizados en el lóbulo temporal (9 derechos y 8 izquierdos). Se realizó extirpación macroscópicamente completa en 17 pacientes y subtotal en los 3 restantes. Se presentaron 4 recidivas que fueron tratadas mediante reintervención, añadiéndose radioterapia en uno de los casos. El tiempo medio de seguimiento fue de 8,5 años (rango 22 meses-14 años), la supervivencia libre de enfermedad a los 5 años fue del 85% y la supervivencia global del 95%. Conclusiones: Las crisis epilépticas, que constituyen el síntoma más frecuente mejoran de forma significativa tras la extirpación quirúrgica. El tratamiento quirúrgico es la primera opción terapéutica en este tipo de tumores, y ante la presencia de resecciones subtotales o recidivas tumorales la mejor indicación de tratamiento es la reintervención. La radioterapia debe reservarse únicamente para las formas malignas (AU)

Introduction: A ganglioglioma is a type of primary central nervous system low grade tumour composed of mixed populations of glial and neuroepithelial elements. They accounts for 0.4 to 2% of all intracranial tumours and appear more commonly in children and young adults. Seizures, which are the most important symptom in these tumours, improve significantly after surgical excision. Methods: Between 1995 and 2008, 20 patients with (12 adults and 8 children) with intracranial ganglioglioma were treated at our hospital. Clinical information obtained by chart review included sex, age at onset of symptoms, clinical history, results of neurological examination, tumour location, CT and MRI appearance, surgical results and follow-up. All patients underwent tumour resection and the extent of surgery was determined from the surgical reports and postoperative imaging studies. Results: The median age of patients was 26.4 years (range, 1-75 years), and the female to male ratio was 1.5:1. Except in one case, all patients had seizures with a median duration before diagnosis of 7.4 years (range 1-29). Seventeen tumours were located in the temporal lobe (9 right and 8 left). Macroscopically complete excision was performed in 17 patients and subtotal in the remaining 3. There were 4 cases of recurrence treated by surgery and radiotherapy being added in one case. The mean follow up was 8.5 years (range 22 months-14 years) and disease free survival at 5 years was 85% and an overall survival of 95%. Conclusions: The seizures, which are the most frequent symptoms, significantly improved after surgical removal. Surgery is the first choice of therapy in these tumours, and in the presence of subtotal resection or tumour recurrence the best indication for treatment is repeat surgery. Radiotherapy should be reserved only for malignant forms (AU)

[Intracranial gangliogliomas. A review of a series of 20 patients]. / Gangliogliomas intracraneales. Revisión de una serie de 20 pacientes.

INTRODUCTION: A ganglioglioma is a type of primary central nervous system low grade tumour composed of mixed populations of glial and neuroepithelial elements. They accounts for 0.4 to 2% of all intracranial tumours and appear more commonly in children and young adults. Seizures, which are the most important symptom in these tumours, improve significantly after surgical excision. METHODS: Between 1995 and 2008, 20 patients with (12 adults and 8 children) with intracranial ganglioglioma were treated at our hospital. Clinical information obtained by chart review included sex, age at onset of symptoms, clinical history, results of neurological examination, tumour location, CT and MRI appearance, surgical results and follow-up. All patients underwent tumour resection and the extent of surgery was determined from the surgical reports and postoperative imaging studies. RESULTS: The median age of patients was 26.4 years (range, 1-75 years), and the female to male ratio was 1.5:1. Except in one case, all patients had seizures with a median duration before diagnosis of 7.4 years (range 1-29). Seventeen tumours were located in the temporal lobe (9 right and 8 left). Macroscopically complete excision was performed in 17 patients and subtotal in the remaining 3. There were 4 cases of recurrence treated by surgery and radiotherapy being added in one case. The mean follow up was 8.5 years (range 22 months-14 years) and disease free survival at 5 years was 85% and an overall survival of 95%. CONCLUSIONS: The seizures, which are the most frequent symptoms, significantly improved after surgical removal. Surgery is the first choice of therapy in these tumours, and in the presence of subtotal resection or tumour recurrence the best indication for treatment is repeat surgery. Radiotherapy should be reserved only for malignant forms.