La fatiga en el daño cerebral sobrevenido / Fatigue in patients with acquired brain damage

La fatiga es un síndrome multidimensional, complejo y frecuente en los pacientes con daño cerebral sobrevenido, influyendo negativamente en el proceso de neurorrehabilitación. Aparece desde etapas tempranas luego de la lesión y puede permanecer en el tiempo, recuperadas o no las secuelas del daño. La fatiga depende de circuitos neuronales superiores y se define como una percepción anómala de sobreesfuerzo. Tiene una prevalencia de 29% a 77% tras el ictus, 18% a 75% tras el traumatismo craneoencefálico (TCE) y 47% a 97% tras tumores cerebrales. La fatiga se asocia a factores como sexo femenino, edad avanzada, familia disfuncional, antecedentes patológicos específicos, estado funcional (p. ej. fatiga previa a la lesión), comorbilidades, estado anímico, discapacidad secundaria y uso de ciertos fármacos. Su estudio se realiza sobre todo a partir de escalas como la Escala de severidad de fatiga (Fatigue Severity Scale). Hoy en día existen avances en herramientas de imagen para su diagnóstico como la resonancia magnética funcional. En cuanto a su tratamiento, no existe aún terapia farmacológica definitiva, sin embargo, existen resultados positivos con terapias dentro de la neurorrehabilitación convencional, terapia lumínica y el uso del neurofeedback, estimulación eléctrica y magnética transcraneal. Esta revisión tiene como objetivo ayudar al profesional dedicado a la neurorrehabilitación a reconocer factores asociados modificables, así como terapias a su alcance para disminuir sus efectos nocivos en el paciente. (AU)

Fatigue is a complex, multidimensional syndrome that is prevalent in patients with acquired brain damage and has a negative impact on the neurorehabilitation process. It presents from early stages after the injury, and may persist over time, regardless of whether sequelae have resolved. Fatigue is conditioned by upper neuronal circuits, and is defined as an abnormal perception of overexertion. Its prevalence ranges from 29% to 77% after stroke, from 18% to 75% after traumatic brain injury, and from 47% to 97% after brain tumours. Fatigue is associated with factors including female sex, advanced age, dysfunctional families, history of specific health conditions, functional status (eg, fatigue prior to injury), comorbidities, mood, secondary disability, and the use of certain drugs. Assessment of fatigue is fundamentally based on such scales as the Fatigue Severity Scale (FSS). Advances have recently been made in imaging techniques for its diagnosis, such as in functional MRI. Regarding treatment, no specific pharmacological treatment currently exists; however, positive results have been reported for some conventional neurorehabilitation therapies, such as bright light therapy, neurofeedback, electrical stimulation, and transcranial magnetic stimulation. This review aims to assist neurorehabilitation professionals to recognise modifiable factors associated with fatigue and to describe the treatments available to reduce its negative effect on patients. (AU)

Fatigue in patients with acquired brain damage.

Fatigue is a complex, multidimensional syndrome that is prevalent in patients with acquired brain damage and has a negative impact on the neurorehabilitation process. It presents from early stages after the injury, and may persist over time, regardless of whether sequelae have resolved. Fatigue is conditioned by upper neuronal circuits, and is defined as an abnormal perception of overexertion. Its prevalence ranges from 29% to 77% after stroke, from 18% to 75% after traumatic brain injury, and from 47% to 97% after brain tumours. Fatigue is associated with factors including female sex, advanced age, dysfunctional families, history of specific health conditions, functional status (eg, fatigue prior to injury), comorbidities, mood, secondary disability, and the use of certain drugs. Assessment of fatigue is fundamentally based on such scales as the Fatigue Severity Scale (FSS). Advances have recently been made in imaging techniques for its diagnosis, such as in functional MRI. Regarding treatment, no specific pharmacological treatment currently exists; however, positive results have been reported for some conventional neurorehabilitation therapies, such as bright light therapy, neurofeedback, electrical stimulation, and transcranial magnetic stimulation. This review aims to assist neurorehabilitation professionals to recognise modifiable factors associated with fatigue and to describe the treatments available to reduce its negative effect on patients.

Combinación de interferones en pacientes con tumores cerebrales de alto grado de malignidad sin opciones terapéuticas / Combination of interferons in patients with high-grade brain tumors without therapeutic options

Introducción: La escasa supervivencia de pacientes con tumores cerebrales de alto grado de malignidad, pese a la existencia de algunas opciones de tratamiento, conduce a la búsqueda de nuevas modalidades terapéuticas. La combinación cubana de interferones alfa y gamma es novedosa y existen evidencias de que aumenta la supervivencia de pacientes con tumores sólidos. Método: Se realizó una investigación clínica para determinar la eficiencia de la combinación en pacientes con tumores cerebrales de alto grado sin opciones terapéuticas. Se incluyeron 40 pacientes tratados en el Hospital “Arnaldo Milián Castro” en el período 2009-2020, se evaluó seguridad y eficacia. Resultados: No se produjeron efectos adversos graves, fueron leves o moderados, y los pacientes se recuperaron. Al año habían fallecido el 8,7 % de los casos del grupo experimental, frente al 70,6 % en el grupo control. La supervivencia global en estadio III fue similar en ambos escenarios y en estadio IV fue superior para el grupo experimental. La posibilidad de sobrevivir para los pacientes que se trataron con la combinación de interferones fue 0,887 veces superior a los casos control. Se produjeron diferencias significativas en la capacidad funcional entre ambos grupos de pacientes. Conclusiones: Se evidenció que la combinación cubana de interferones es segura y eficaz para el tratamiento de tumores cerebrales de alto grado de malignidad sin opciones terapéuticas, lo que la convierte en una opción eficiente en este escenario clínico. (AU)

Introduction: The poor survival of patients with high-grade malignancy brain tumors, despite the existence of some treatment options, leads to the search for new therapeutic modalities. The cuban combination of alpha and gamma interferons is novel and there is evidence that it increases the survival of patients with solid tumors. Method: A clinical investigation was conducted to determine the efficiency of the combination in patients with high-grade brain tumors without therapeutic options. 40 patients treated at the “Arnaldo Milián Castro” Hospital in the period 2009-2020 were included, safety and efficacy were evaluated. Results: No serious adverse events occurred, events were mild or moderate, expected, and patients recovered. After one year, 8.7 % of the cases in the experimental group had died, compared to 70.6 % in the control group. Overall survival in stage III was similar in both scenarios and in stage IV it was higher for the experimental group. The chance of survival for the patients who were treated with the combination of interferons was 0.887 times higher than the control cases. There were significant differences in functional capacity between both groups of patients. Conclusions: It was evidenced that the Cuban combination of interferons is safe and effective for the treatment of high-grade malignancy brain tumors without therapeutic options, which makes it an efficient option in this clinical scenario. (AU)

Consenso sobre el tratamiento neuroquirúrgico de las metástasis intracraneales elaborado por el Grupo de Trabajo de Neurooncología (GTNO) de la Sociedad Española de Neurocirugía / Brain metastasis treatment guidelines: Consensus by the Spanish Society of Neurosurgery Tumor Section

Las metástasis cerebrales (MC) son tumores que se forman a partir de una célula tumoral originada en otro órgano y que a través de la sangre llega al cerebro donde es capaz de crecer e invadir los tejidos vecinos, como meninges y hueso. En la mayor parte de los pacientes existe un tumor conocido cuando se diagnostica la lesión cerebral, aunque es posible que el tumor del cerebro sea el primer hallazgo antes de que se tenga evidencia de la patología oncológica en otro lugar del organismo. Por este motivo, el neurocirujano debe conocer el manejo que ha demostrado mayor beneficio para estos sujetos, de manera que se agilicen y optimicen los tratamientos. Concretamente, en este documento se desarrollarán, entre otros temas: la selección del paciente oncológico candidato a la resección quirúrgica y el papel del neurocirujano en el equipo multidisciplinar, la importancia del diagnóstico inmunohistológico y molecular, técnicas quirúrgicas y de RT, actualización de tratamientos de quimioterapia e inmunoterapia y algoritmos de manejo en MC. Con este manuscrito de consenso, el Grupo de Tumores de la Sociedad Española de Neurocirugía (GT- SENEC) expone las cuestiones neuroquirúrgicas más relevantes y los aspectos fundamentales para armonizar el tratamiento multidisciplinar, sobre todo con las especialidades médicas que estén tratando o vayan a abordar a estos pacientes (AU)

Brain metastases are tumors that arise from a tumor cell originated in another organ reaching the brain through the blood. In the brain this tumor cell is capable of growing and invading neighboring tissues, such as the meninges and bone. In most patients a known tumor is present when the brain lesion is diagnosed, although it is possible that the first diagnose is the brain tumor before there is evidence of cancer elsewhere in the body. For this reason, the neurosurgeon must know the management that has shown the greatest benefit for brain metastasis patients, so treatments can be streamlined and optimized. Specifically, in this document, the following topics will be developed: selection of the cancer patient candidate for surgical resection and the role of the neurosurgeon in the multidisciplinary team, the importance of immunohistological and molecular diagnosis, surgical techniques, radiotherapy techniques, treatment updates of chemotherapy and immunotherapy and management algorithms in brain metastases. With this consensus manuscript, the tumor group of the Spanish Society of Neurosurgery (GT-SENEC) exposes the most relevant neurosurgical issues and the fundamental aspects to harmonize multidisciplinary treatment, especially with the medical specialties that are treating or will treat these patients (AU)

Brain metastasis treatment guidelines: consensus by the Spanish Society of Neurosurgery Tumor Section.

Brain metastases are tumors that arise from a tumor cell originated in another organ reaching the brain through the blood. In the brain this tumor cell is capable of growing and invading neighboring tissues, such as the meninges and bone. In most patients a known tumor is present when the brain lesion is diagnosed, although it is possible that the first diagnose is the brain tumor before there is evidence of cancer elsewhere in the body. For this reason, the neurosurgeon must know the management that has shown the greatest benefit for brain metastasis patients, so treatments can be streamlined and optimized. Specifically, in this document, the following topics will be developed: selection of the cancer patient candidate for surgical resection and the role of the neurosurgeon in the multidisciplinary team, the importance of immunohistological and molecular diagnosis, surgical techniques, radiotherapy techniques, treatment updates of chemotherapy and immunotherapy and management algorithms in brain metastases. With this consensus manuscript, the tumor group of the Spanish Society of Neurosurgery (GT-SENEC) exposes the most relevant neurosurgical issues and the fundamental aspects to harmonize multidisciplinary treatment, especially with the medical specialties that are treating or will treat these patients.

Hemorrhagic presentation of previously silent brain tumors / Presentación hemorrágica de tumores cerebrales anteriormente silenciosos

Introduction and objectives Acute presentation with intracranial hemorrhage owing to a previously silent brain tumor (BT) is rare. Although any BT can bleed, the frequency and type of bleeding varies across tumor types. Materials and methods We aimed to retrospectively review our experience with 55 patients with BTs presenting with ICH. Results Signs of increased intracranial pressure were the most common symptoms. The temporal lobe was the most common lesion site (n=22). Hemorrhages were mainly confined to the tumor margins (HCTs) (n=34). Extensive intraparenchymal hemorrhages (EIHs) were mainly associated with moderately/severely decreased levels of consciousness (LOCs) (n=15/16). High-grade glioma (HGGT) (n=25) was the leading pathological diagnosis followed by metastasis (MBT) (n=16/55). The hemorrhage type was associated with the pathological diagnosis of the tumor. Patients with HGGT (n=19/25) and MBT (n=9/16) mainly presented with HCTs, whereas low-grade gliomas (LGGT) primarily caused EIHs (n=6/7). Conclusions Hemorrhagic presentation is a rare occurrence in BTs. Among all, MBT and HGGT are responsible for majority of the cases. Importantly, despite their relatively benign characteristics, LGGTs mainly result in extensive parenchymal destruction once they bleed. Maximum surgical resection of hemorrhagic BTs and decompression of the affected brain regions followed by histological confirmation of the diagnosis should be the main goals of treatment in cases with hemorrhagic BTs (AU)

Introducción y objetivos La presentación aguda con hemorragia intracraneal debida a un tumor cerebral (BT) anteriormente silencioso es rara. A pesar de que cualquier BT puede sangrar, la frecuencia y el tipo de sangrado varían según el tipo de tumor. Materiales y métodos Nuestro objetivo fue reexaminar retrospectivamente nuestra experiencia con 55 pacientes con los BT que presentaban HIC. Resultados Los síntomas más comunes fueron signos de aumento de la presión intracraneal. El lóbulo temporal fue el sitio de lesión más común (n=22). Las hemorragias se limitaron especialmente a los márgenes tumorales (HCT) (n=34). Las hemorragias intraparenquimatosas extensas (HIE) se asociaron mayormente con niveles de conciencia moderada/severamente disminuidos (LOC) (n=15/16). El glioma de alto grado (HGGT) (n=25) fue el principal diagnóstico patológico después de la metástasis (MBT) (n=16/55). El tipo de hemorragia se asoció con el diagnóstico patológico del tumor. Los pacientes con HGGT (n=19/25) y MBT (n=9/16) presentaron mayormente con HCT, mientras que los gliomas de bajo grado (LGGT) causaron principalmente HIE (n=6/7). Conclusiones La presentación hemorrágica es una ocurrencia rara en los BT. Entre todos, MBT y HGGT son responsables de la mayoría de los casos. Más importante aún, pese a sus características relativamente benignas, los LGGT resultan mayormente una destrucción extensa del parénquima una vez que sangran. La resección quirúrgica máxima de BT hemorrágicos y la descompresión de las regiones cerebrales afectadas con la confirmación histológica del diagnóstico deben ser los objetivos principales del tratamiento en casos con BT hemorrágicos (AU)

Consenso sobre guías de tratamiento de gliomas de bajo grado elaborado por el grupo de tumores de la SENEC / Low grade gliomas guide-lines elaborated by the tumor section of Spanish Society of Neurosurgery

Los gliomas de bajo grado (Low Grade Gliomas, LGG) del adulto son tumores que se originan a partir de las células gliales del cerebro y cuyo manejo implica gran controversia a día de hoy, comenzando desde el diagnóstico, hasta el tratamiento y seguimiento posterior de estos pacientes. Es por ello que el grupo de tumores de la Sociedad Española de Neurocirugía (GT-SENEC) ha llevado a cabo una reunión de consenso, en la que se han debatido las cuestiones neuroquirúrgicas más relevantes, llegando a recomendaciones basadas en la mejor evidencia científica. Con el fin de obtener el máximo beneficio a estos tratamientos se debe hacer una valoración individualizada de cada paciente por un equipo multidisciplinar, constituido por aquellas especialidades involucradas tanto en el diagnóstico como en el tratamiento. El objetivo de este trabajo es elaborar unas recomendaciones de tratamiento para los pacientes con LGG, para lo cual un experto en cada campo ha descrito lo más relevante de dicha área basado tanto en su experiencia como en la literatura. Se han desarrollado los apartados más relevantes en el manejo de los LGG basados en la mejor evidencia publicada. A pesar de que existe controversia en algunos aspectos del manejo de los LGG, cada vez hay más datos para poder hacer recomendaciones de tratamiento consensuadas. El neurocirujano debe conocer las distintas opciones de tratamientos, sus indicaciones y riesgos para poder participar activamente en la toma de decisiones y poder ofrecer un tratamiento neuroquirúrgico oportuno a cada situación (AU)

Adult low-grade gliomas (Low Grade Gliomas, LGG) are tumors that originate from the glial cells of the brain and whose management involves great controversy, starting from the diagnosis, to the treatment and subsequent follow-up. For this reason, the Tumor Group of the Spanish Society of Neurosurgery (GT-SENEC) has held a consensus meeting, in which the most relevant neurosurgical issues have been discussed, reaching recommendations based on the best scientific evidence. In order to obtain the maximum benefit from these treatments, an individualized assessment of each patient should be made by a multidisciplinary team. Experts in each LGG treatment field have briefly described it based in their experience and the reviewed of the literature. Each area has been summarized and focused on the best published evidence. LGG have been surrounded by treatment controversy, although during the last years more accurate data has been published in order to reach treatment consensus. Neurosurgeons must know treatment options, indications and risks to participate actively in the decision making and to offer the best surgical treatment in every case (AU)

Hemorrhagic presentation of previously silent brain tumors.

INTRODUCTION AND OBJECTIVES: Acute presentation with intracranial hemorrhage owing to a previously silent brain tumor (BT) is rare. Although any BT can bleed, the frequency and type of bleeding varies across tumor types. MATERIALS AND METHODS: We aimed to retrospectively review our experience with 55 patients with BTs presenting with ICH. RESULTS: Signs of increased intracranial pressure were the most common symptoms. The temporal lobe was the most common lesion site (n=22). Hemorrhages were mainly confined to the tumor margins (HCTs) (n=34). Extensive intraparenchymal hemorrhages (EIHs) were mainly associated with moderately/severely decreased levels of consciousness (LOCs) (n=15/16). High-grade glioma (HGGT) (n=25) was the leading pathological diagnosis followed by metastasis (MBT) (n=16/55). The hemorrhage type was associated with the pathological diagnosis of the tumor. Patients with HGGT (n=19/25) and MBT (n=9/16) mainly presented with HCTs, whereas low-grade gliomas (LGGT) primarily caused EIHs (n=6/7). CONCLUSIONS: Hemorrhagic presentation is a rare occurrence in BTs. Among all, MBT and HGGT are responsible for majority of the cases. Importantly, despite their relatively benign characteristics, LGGTs mainly result in extensive parenchymal destruction once they bleed. Maximum surgical resection of hemorrhagic BTs and decompression of the affected brain regions followed by histological confirmation of the diagnosis should be the main goals of treatment in cases with hemorrhagic BTs.

Low grade gliomas guide-lines elaborated by the tumor section of Spanish Society of Neurosurgery.

Adult low-grade gliomas (Low Grade Gliomas, LGG) are tumors that originate from the glial cells of the brain and whose management involves great controversy, starting from the diagnosis, to the treatment and subsequent follow-up. For this reason, the Tumor Group of the Spanish Society of Neurosurgery (GT-SENEC) has held a consensus meeting, in which the most relevant neurosurgical issues have been discussed, reaching recommendations based on the best scientific evidence. In order to obtain the maximum benefit from these treatments, an individualised assessment of each patient should be made by a multidisciplinary team. Experts in each LGG treatment field have briefly described it based in their experience and the reviewed of the literature. Each area has been summarized and focused on the best published evidence. LGG have been surrounded by treatment controversy, although during the last years more accurate data has been published in order to reach treatment consensus. Neurosurgeons must know treatment options, indications and risks to participate actively in the decision making and to offer the best surgical treatment in every case.

Nanocannabinoides como terapia frente a glioma / Nannocannabinoids for brain tumor drug delivery

Las patologías cerebrales representan un desafío terapéutico por la restricción al paso de fármacos a través de la barrera hematoencefálica. Por ello, actualmente se persigue diseñar transportadores de fármacos capaces de atravesar de manera eficiente el endotelio cerebral tras su administración intravenosa. Sin embargo, el impacto traslacional de la nanomedicina es aún discreto. Sin duda, la transición de un desarrollo empírico hacia un diseño racional adecuado a las necesidades terapéuticas concretas en cada caso aumentará las posibilidades de éxito.Bajo esta premisa y aprovechando tanto el tropismo cerebral como la actividad antiproliferativa del cannabidiol, y a fin de contribuir al diseño racional de nanocápsulas dirigidas para el tratamiento de gliomas, hemos evaluado la influencia de distintos parámetros en su comportamiento in vitro e in vivo. Efectivamente, hemos demostrado que tanto el paso a través de barrera hematoencefálica como la captación por células de glioma, así como la velocidad de liberación de fármacos pueden modularse variando su tamaño de partícula. El método térmico de inversión de fases posibilita la obtención de nanocápsulas bajo demanda en términos de tamaño gracias al modelo matemático lineal en una variable aquí descrito.Además, hemos desarrollado una novedosa estrategia de vectorización con cannabidiol (que incluso supera a otras que ya se encuentran en ensayos clínicos). Asimismo, las nanocápsulas sirven como transportadores de liberación prolongada del cannabidiol, superando así sus problemas de formulación que venían limitando su potencial terapéutico.En conjunto, las nanocápsulas lipídicas, cargadas y funcionalizadas con cannabidiol, constituyen prometedores candidatos para el tratamiento de gliomas. (AU)

Brain diseases are a major health challenge as brain drug delivery is truly hindered by the blood-brain barrier. Therefore, targeted drug nanocarriers arise as an alternative to achieve efficient transport across the brain endothelium following minimally-invasive intravenous injection. However, the global translational impact of nanomedicine remains modest. Certainly, the transition from empirical development towards a rational design tailored to the specific disease needs is likely to improve the chances of success.Under this assumption and taking advantage of both the natural brain tropism and the antiproliferative activity of cannabidiol, to contribute to the rational design of targeted nanocapsules for glioma therapy, we have thoroughly screened the influence of distinct parameters on their in vitro and in vivo behaviour. Effectively, we have demonstrated that both the brain and glioma targeting ability and the drug release rate can be tailored by varying the particle size of the nanocapsules. This fine size-tailoring can be achieved by the phase inversion temperature method thanks to the hereindescribed linear univariate mathematical model as a function of the oily phase/surfactant mass ratio.Moreover, we have introduced, on the one hand, a pioneering brain tumor targeting strategy with cannabidiol (with better targeting properties than other strategies that have already reached the clinical trials stage) and, on the other hand, nanocapsules as extendedrelease carriers of cannabidiol to overcome the formulation problems that have traditionally constrained its therapeutic potential.Altogether, small lipid nanocapsules loaded and functionalized with cannabidiol arise as promising dually-targeted candidates for intravenous treatment of glioma. (AU)

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature / Linfoma de Hodgkin primario del sistema nervioso central. Presentación de un caso y revisión de la literatura

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS–HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS. In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS–HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy±chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma. We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS–HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented (AU)

La afectación del sistema nervioso central (SNC) en pacientes con diagnóstico de linfoma de Hodgkin (LH) sistémico es una complicación muy poco frecuente, aunque bien definida. Sin embargo, el LH primario del SNC es una entidad extremadamente rara, cuyo diagnóstico precisa la identificación de un patrón morfológico e inmunohistoquímico específico, así como la afectación aislada del SNC. Pese a contar con una casuística muy limitada (apenas una veintena de casos publicados en la literatura) la bibliografía disponible coincide en que el LH con afectación primaria y aislada del SNC, cuando es tratado con cirugía y tratamiento adyuvante (radioterapia±quimioterapia), parece tener un mejor pronóstico que aquellos casos en los que la afectación del SNC se produce en el contexto de un LH sistémico o en el contexto de un linfoma no Hodgkin. En este artículo se presenta el caso de una mujer de 55 años con diagnóstico histopatológico de LH primario del SNC. La paciente fue sometida a una exéresis completa de la lesión y a tratamiento adyuvante con quimioterapia intratecal y radioterapia holocraneal, con una supervivencia libre de enfermedad hasta la fecha de 14 meses. Se presenta asimismo, la revisión de la literatura disponible (AU)

Variante amelanótica en melanoma cerebral primario: a propósito de un caso / Primary intracranial melanoma, amelanotic variant: Case report

El melanoma cerebral primario es un tumor muy infrecuente (0,07% de las neoplasias primarias del SNC). Generalmente muestra un abundante contenido en melanina, y solo en contadas ocasiones se han descrito variantes hipoamelanóticas. Presentamos el caso de una paciente con clínica de cefalea, paresia braquial izquierda y síndrome lobar frontal. La RM mostró una masa frontal derecha con captación homogénea de contraste. Como tratamiento, se realizó una resección quirúrgica completa. El estudio anatomopatológico fue diagnóstico para melanoma, con muy escaso contenido en melanina y alto índice proliferativo. Se realizó un estudio de extensión exhaustivo para descartar otra localización primaria. Debido a varias complicaciones intercurrentes, la paciente evolucionó desfavorablemente, sin llegar a recibir otros tratamientos. La variante amelanótica de los melanomas cerebrales primarios no ha sido descrita con detalle previamente. Repasamos la literatura al respecto y discutimos los detalles de manejo y diagnóstico de esta entidad clínica (AU)

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described. We introduce the case of a female patient with headache, left brachial paresis and frontal lobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment. The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity (AU)

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature.

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS-HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS. In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS-HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy±chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma. We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS-HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented.

Efecto de la alteración de los niveles de expresión de microARN neurogénicos y su implicación en la agresividad de glioblastomas localizados en la región paraventricular / The effects of altered neurogenic microRNA levels and their involvement in the aggressiveness of periventricular glioblastoma

Introducción: El glioblastoma multiforme es el tumor cerebral primario más común y con el pronóstico más desfavorable del sistema nervioso central. A pesar de los numerosos estudios y avances en medicina, este sigue siendo letal, con una esperanza de vida promedio de 15 meses posteriores a la quimiorradioterapia.DesarrolloRecientemente, se han estudiado diversos factores asociados al diagnóstico y el pronóstico de pacientes con glioblastoma, como la localización tumoral, principalmente la zona subventricular; una de las áreas neurogénicas más activas del cerebro humano adulto. Los pacientes con glioblastoma asociados a esta zona en particular presentan generalmente una mayor agresividad, lo que resulta en un pronóstico desfavorable y una menor esperanza de vida. Actualmente, se ha profundizado en el estudio de los microARN, los cuales reflejan patrones de expresión distintos en condiciones fisiológicas o fisiopatológicas. Está reportado que los niveles de expresión de ciertos microARN, principalmente aquellos relacionados a procesos neurogénicos, se ven desregulados en eventos oncogénicos, favoreciendo así la gliomagénesis y la agresividad tumoral. En la presente revisión se discuten algunos de los microARN más importantes implicados en procesos neurogénicos de la zona subventricular y su asociación con la agresividad del glioblastoma.ConclusionesLa regulación y función de los microARN desempeña un rol importante en el desarrollo y la progresión del glioblastoma; en consecuencia, la comprensión de las alteraciones de los microARN implicados en la diferenciación, así como en la maduración neural y glial, podrían ayudar a entender mejor las características malignas del glioblastoma. (AU)

Introduction: Glioblastoma multiforme is the most common primary brain tumour, with the least favourable prognosis. Despite numerous studies and medical advances, it continues to be lethal, with an average life expectancy of 15 months after chemo-radiotherapy.DevelopmentRecent research has addressed several factors associated with the diagnosis and prognosis of glioblastoma; one significant factor is tumour localisation, particularly the subventricular zone, which represents one of the most active neurogenic niches of the adult human brain. Glioblastomas in this area are generally more aggressive, resulting in unfavourable prognosis and a shorter life expectancy. Currently, the research into microRNAs (miRNA) has intensified, revealing different expression patterns under physiological and pathophysiological conditions. It has been reported that the expression levels of certain miRNAs, mainly those related to neurogenic processes, are dysregulated in oncogenic events, thus favouring gliomagenesis and greater tumour aggressiveness. This review discusses some of the most important miRNAs involved in subventricular neurogenic processes and their association with glioblastoma aggressiveness.ConclusionsMiRNA regulation and function play an important role in the development and progression of glioblastoma; understanding the alterations of certain miRNAs involved in both differentiation and neural and glial maturation could help us to better understand the malignant characteristics of glioblastoma. (AU)

Preoperative 3D volume reconstruction of the posterior wall of the sphenoid sinus with Horos: A free, simple and reliable tool in endoscopic endonasal trans-sphenoidal surgery / Reconstrucción volumétrica 3D preoperatoria de la pared posterior del seno esfenoidal con Horos: una herramienta gratuita, sencilla y fiable en la cirugía endoscópica endonasal transesfenoidal

BackgroundThe purpose of the study was to create computer-aided design models of the sphenoid sinus with a free-source software in order to perform a preoperative planning during trans-sphenoidal endoscopic surgery (TES) and clarify the three-dimensional anatomical features of the sphenoid sinus and its surrounding structures.MethodsFor each patient a 3D volume rendering of the sphenoid sinus was obtained from a thin slice head and maxilla-facial CT scan using a free-source DICOM viewer. The 3D models obtained preoperatively were examined preoperatively by six neurosurgeons in order to identify the boundaries of the sella.ResultsFor the main anatomical landmark, all of the observers were able to recognize the anatomical structure at a rate ranging from 80 to 98%, 28 to 60% and 25 to 58% for expert (n=3), inexpert (n=3) and the entire group of observers (n=6), respectively. The analysis of the data shows that both observation groups presented a lower recognition rate of the following parameters: right and left medial and lateral optocarotid recesses and tumor prominence, however, the sellar prominence, clival recess, planum sphenoidalis, right and left ICA prominence, right and left optic prominences represent the main anatomical landmarks to be recognized during TES immediately before the opening of the sellar floor.ConclusionsThe use of a preoperative 3D imaging is not in itself a novelty in the literature, however the fact that a simple tool obtained with a free-source software like Horos can represent a considerable help in surgical practice without resorting to the use of more complex software and expensive represents the real utility of this work (AU)

AntecedentesEl objetivo del estudio es crear modelos de diseño asistido por ordenador del seno esfenoidal con software de código abierto para realizar la planificación preoperatoria durante la cirugía transesfenoidal endoscópica (TES) y dilucidar las características anatómicas tridimensionales del seno esfenoidal y las estructuras que lo rodean.MétodosPara cada paciente, se obtuvo una representación volumétrica en 3D del seno esfenoidal, a partir de una tomografía computarizada maxilofacial de corte fino utilizando un visor DICOM de fuente libre. Los modelos 3D obtenidos antes de la cirugía fueron revisados por seis neurocirujanos para identificar los límites de la silla turca.ResultadosPara el principal punto de referencia anatómico, todos los observadores fueron capaces de reconocer la estructura anatómica en un rango de 80 a 98%, 28 a 60% y 25 a 58% para los experimentados (n=3), los inexpertos (n=3) y todo el grupo de observadores (n=6), respectivamente. El análisis de los datos muestra que ambos grupos de observación presentaron una tasa de reconocimiento menor de los siguientes parámetros: recesos optocarotídeos medial y lateral izquierdo y prominencia tumoral, sin embargo, prominencia selar, receso clival, plano esfenoidal, prominencia ICA derecha e izquierda. Las prominencias ópticas derecha e izquierda representan los principales puntos de referencia anatómicos que deben reconocerse durante la TES inmediatamente antes de la apertura del suelo selar.ConclusionesEl uso de una imagen 3D preoperatoria no es en sí mismo una novedad en la literatura, sin embargo, el hecho de que una simple herramienta obtenida con un software de código abierto como Horos pueda representar una importante ayuda en la práctica quirúrgica, sin tener que recurrir al uso de un software más complejo y costoso, representa la verdadera utilidad de este trabajo (AU)

Myxofibrosarcoma: Another mimicker of meningioma / Mixofibrosarcoma: otro imitador del meningioma

Primary intracranial malignant fibrous histiocytoma (MFH), or myxofibrosarcoma, is an extremely rare condition, with only a few cases reported in the literature. We report a case of a dural-based myxofibrosarcoma in a previously healthy 42-year-old man that was initially presumed to be an atypical meningioma. The findings based on conventional and advanced magnetic resonance sequences, including diffusion-weighted imaging, perfusion weighted imaging and proton magnetic resonance spectroscopy, as well as histopathological aspects, are discussed (AU)

El histiocitoma fibroso maligno intracraneal primario (HFM) o mixofibrosarcoma, es una condición extremadamente rara, con solo unos pocos casos reportados en la literatura. Presentamos un caso de mixofibrosarcoma de base dural en un varón de 42 años, previamente sano, que inicialmente se presumió que era un meningioma atípico. Se discuten los hallazgos basados en secuencias de resonancia magnética convencionales y avanzadas, que incluyen imágenes ponderadas por difusión, imágenes ponderadas por perfusión y espectroscopía de resonancia magnética de protones, así como aspectos histopatológicos (AU)

Preoperative 3D volume reconstruction of the posterior wall of the sphenoid sinus with Horos: A free, simple and reliable tool in endoscopic endonasal trans-sphenoidal surgery.

BACKGROUND: The purpose of the study was to create computer-aided design models of the sphenoid sinus with a free-source software in order to perform a preoperative planning during trans-sphenoidal endoscopic surgery (TES) and clarify the three-dimensional anatomical features of the sphenoid sinus and its surrounding structures. METHODS: For each patient a 3D volume rendering of the sphenoid sinus was obtained from a thin slice head and maxilla-facial CT scan using a free-source DICOM viewer. The 3D models obtained preoperatively were examined preoperatively by six neurosurgeons in order to identify the boundaries of the sella. RESULTS: For the main anatomical landmark, all of the observers were able to recognize the anatomical structure at a rate ranging from 80 to 98%, 28 to 60% and 25 to 58% for expert (n=3), inexpert (n=3) and the entire group of observers (n=6), respectively. The analysis of the data shows that both observation groups presented a lower recognition rate of the following parameters: right and left medial and lateral optocarotid recesses and tumor prominence, however, the sellar prominence, clival recess, planum sphenoidalis, right and left ICA prominence, right and left optic prominences represent the main anatomical landmarks to be recognized during TES immediately before the opening of the sellar floor. CONCLUSIONS: The use of a preoperative 3D imaging is not in itself a novelty in the literature, however the fact that a simple tool obtained with a free-source software like Horos can represent a considerable help in surgical practice without resorting to the use of more complex software and expensive represents the real utility of this work.

Myxofibrosarcoma: Another mimicker of meningioma.

Primary intracranial malignant fibrous histiocytoma (MFH), or myxofibrosarcoma, is an extremely rare condition, with only a few cases reported in the literature. We report a case of a dural-based myxofibrosarcoma in a previously healthy 42-year-old man that was initially presumed to be an atypical meningioma. The findings based on conventional and advanced magnetic resonance sequences, including diffusion-weighted imaging, perfusion weighted imaging and proton magnetic resonance spectroscopy, as well as histopathological aspects, are discussed.

Factores pronósticos asociados a la supervivencia de pacientes con tumores astrocíticos / Prognostic factors associated with the survival of patients with astrocytic tumors / Fatores prognósticos associados à sobrevida de pacientes com tumores astrocíticos

RESUMEN Introducción: Los tumores cerebrales son un grupo heterogéneo dada las diferentes líneas celulares que los originan. Los tumores cerebrales más frecuentes son los que se derivan de la glía, siendo los astrocitomas los más comunes. Objetivo: Sistematizar los hallazgos de los estudios que examinaron múltiples factores pronósticos de supervivencia en pacientes con tumores cerebrales tipo astrocitoma. Método: Se realizó una revisión sistemática de estudios observacionales, analíticos, prospectivos. La búsqueda bibliográfica se efectuó en las bases de datos electrónicas Medline, SciELO, LILACS, PubMed y Google Académico, con los siguientes términos descriptores: edad, sexo, factores pronósticos, cáncer cerebral, astrocitoma. Resultados: En la búsqueda se hallaron 136 452 artículos que estaban distribuidos en diferentes bases de datos. Al aplicar los criterios de elegibilidad quedaron para el análisis 13 artículos. En el caso de los factores pronósticos dependientes de la lesión, tumor o enfermedad, en la mayoría de las investigaciones se mostró una tendencia a evaluar el grado histológico y la histología de la lesión. En el caso de los pronósticos dependientes del paciente existió una mayor inclinación hacia la edad, el sexo y la sintomatología presentada, mientras que en los dependientes del tratamiento se mostraron indistintamente las modalidades de tratamiento sugeridas a los pacientes: cirugía, quimioterapia y radioterapia. Conclusiones: En esta investigación se presentaron los principales factores pronósticos en pacientes con tumores astrocíticos contenidos en las literaturas referentes al tema, que se agrupan en factores pronósticos dependientes del tumor, el paciente y el tratamiento. Estos deben ser tenidos en cuenta para evaluar al paciente con estas lesiones tumorales cerebrales.

ABSTRACT Introduction: Brain tumors are a heterogeneous group given the different cells that originate them. The most frequent brain tumors are those derived from the glia, with astrocytomas being the most common. Objective: To systematize the findings of studies that examined multiple prognostic factors for survival in patients with astrocytoma-type brain tumors. Method: a systematic review of observational, analytical, prospective studies was carried out. The bibliographic search was carried out in the electronic databases Medline, SciELO, LILACS, PubMed and Google Scholar, with the following descriptive terms: age, sex, prognostic factors, brain cancer, astrocytoma. Results: The search found 136,452 articles that were distributed in different databases. When applying the eligibility criteria, 13 articles remained for analysis. In the case of prognostic factors dependent on the lesion, tumor or disease, most researches showed a tendency to evaluate the histologic grade and the histology of the lesion. In the case of the patient-dependent prognoses, there was a greater inclination towards age, sex and the symptoms presented, while in those dependent on the treatment, the treatment modalities suggested to the patients were shown indistinctly: surgery, chemotherapy and radiotherapy. Conclusions: In this research, the main prognostic factors contained in the literature about patients with astrocytic tumors were presented, which are grouped into prognostic factors depending on the tumor, the patient and the treatment. These must be taken into account to evaluate the patient with these brain tumor lesions.

RESUMO Introdução: Os tumores cerebrais são um grupo heterogêneo devido às diferentes linhagens celulares que os originam. Os tumores cerebrais mais frequentes são os derivados da glia, sendo os astrocitomas os mais comuns. Objetivo: Sistematizar os achados de estudos que examinaram múltiplos fatores prognósticos de sobrevida em pacientes com tumores cerebrais do tipo astrocitoma. Método: Foi realizada uma revisão sistemática de estudos observacionais, analíticos e prospectivos. A busca bibliográfica foi realizada nas bases de dados eletrônicas Medline, SciELO, LILACS, PubMed e Google Acadêmico, com os seguintes termos descritivos: idade, sexo, fatores prognósticos, câncer cerebral, astrocitoma. Resultados: A busca encontrou 136.452 artigos que foram distribuídos em diferentes bases de dados. Ao aplicar os critérios de elegibilidade, restaram 13 artigos para análise. No caso de fatores prognósticos dependentes da lesão, tumor ou doença, a maioria das investigações mostrou uma tendência a avaliar o grau histológico e a histologia da lesão. No caso dos prognósticos paciente-dependentes, houve maior inclinação para a idade, sexo e os sintomas apresentados, enquanto nos dependentes do tratamento, as modalidades de tratamento sugeridas aos pacientes foram mostradas indistintamente: cirurgia, quimioterapia e radioterapia. Conclusões: Nesta pesquisa foram apresentados os principais fatores prognósticos em pacientes com tumores astrocíticos contidos na literatura sobre o assunto, os quais são agrupados em fatores prognósticos dependendo do tumor, do paciente e do tratamento. Estes devem ser levados em consideração para avaliar o paciente com essas lesões tumorais cerebrais.

[Lipomatous ependymomas of the posterior fossa. An infrequent and little-known subtype. A case report and review of the literature]. / Ependimomas lipomatosos de la fosa posterior. Un subtipo muy infrecuente y poco conocido. Presentación de caso y revisión de la literatura.

Ependymomas are well defined glial tumours composed of uniform small cells with round nuclei in a fibrillar matrix. They have characteristic perivascular acellular areas (pseudorosettes) and, in some cases, ependymal rosettes. The three most well-known histological phenotypes are papillary, clear-cell and tanycytic. The WHO classification includes rare cases of ependymoma with lipomatous metaplasia. Lipomatous ependymomas of the posterior fossa are extremely rare; we only found 7reports of cases in adults. They usually arise in the fourth ventricle and may extend into the cerebellum, when they often show extensive vacuolization, pushing the nucleus to the periphery and giving rise to a signet-ring cell appearance. Radiologically, there are few findings characteristic of these tumours. Immunohistochemistry is essential to differentiate this subtype from other more common lesions, such as metastatic adenocarcinoma, especially from breast, intestine and kidney.