Epileptic encephalopathies secondary to hypothalamic hamartomas treated with radiosurgery: A case series.

OBJECTIVE: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications. METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®. RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported. SIGNIFICANCE: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.

Características clínicas y patológicas de pacientes con tumores del sistema nervioso central en un centro en Suramérica desde 2010 hasta 2015 / Clinico-pathological characteristics of central nervous system tumors in a South American center from 2010 to 2015

Resumen Objetivo: Caracterizar los pacientes diagnosticados con tumores del sistema nervioso central en el Instituto Neurológico de Colombia durante el periodo 2010-2015. Métodos: Estudio descriptivo retrospectivo. Para los tumores primarios se usó la clasificación de la Organización Mundial de la Salud y para los metastásicos se usó la Clasificación Internacional de Enfermedades en Oncología. Resultados: Se identificaron 288 pacientes, 194 de ellos con tumores primarios y 94 tumores metastásicos. No se lograron clasificar los tumores primarios en el 23,7 % de los casos y para los metastásicos no se obtuvo la clasificación morfológica (histológica) en el 35,1 % de los casos. Los hombres presentaron con mayor frecuencia tumores de comportamiento maligno tipo glioblastoma NEO (no especificado de otra manera) (14,9 %) y en las mujeres predominaron los tumores de comportamiento benigno tipo meningioma (23,2 %). En mayores de 65 años, tanto el glioblastoma NEO como el meningioma fueron más frecuentes en mujeres con 17,4 % y 28,3 %, respectivamente. Entre los pacientes con tumores metastásicos, de acuerdo con la clasificación topográfica, los tumores primarios más frecuentes se localizaron en pulmón (39,4 %) y mama (17 %). No se identificó el sitio primario de metástasis en el 11,7 % de los casos. La histología más comúnmente identificada fue el adenocarcinoma (14,9 %), seguido del carcinoma (8,5 %). Conclusiones: Para una vigilancia efectiva de la enfermedad es necesario realizar un monitoreo epidemiológico y clínico de tumores primarios y metastásicos, mediante el uso de registros institucionales de cáncer, incluyendo datos topográficos, histológicos y moleculares, según disponibilidad.

Abstract Objective: The aim of this study was to characterize patients with a diagno- sis of a central nervous system (CNS) tumors at the Instituto Neurologico de Colombia during the period between 2010 to 2015. Methods: A retrospective descriptive study was conducted. The Classification of the World Health Organization was used for CNS primary tumors whereas the International Classification of Diseases for oncology (ICD-O) was used for CNS metastatic tumors. Results: 288 patients were identified, 194 of them with primary tumors of CNS and 94 with metastatic tu- mors from systemic cancer. It was not possible to classify primary tumors in 23.7% of the cases and regarding the metastatic tumors it was not possible to obtain the classification in 35.1 % of the cases. Men presented more frequently tumors of ma- lignant behavior such as glioblastoma NOS (not otherwise specified) (14.9 %) while in women benign behavior tumors such as meningioma predominated (23.2 %). For population older than 65 years old, both glioblastoma NOS and meningioma were more frequent in women with 17.4 % and 28.3 %, respectively. In patients with CNS metastatic tumors, according to the topographic classification, the most frequent primary tumors were lung (39.4 %), followed by breast (17 %). Its origin was not iden- tified in 11.7 % of the cases. The most identified histology was adenocarcinoma (14.9 %), followed by carcinoma (8.5 %). Conclusion: For disease surveillance, it is necessary to complete epidemiological and clinical monitoring of primary and metastatic tumors of the CNS by using institutional cancer registries including topographic, histological and molecular data according to availability.