[Tuberous sclerosis complex: diagnosis and current treatment]. / Complejo de esclerosis tuberosa: diagnóstico y tratamiento actual.

Tuberous sclerosis complex is an autosomal dominant genetic multisystemic disorder caused primarily by mutations in one of the two tumor suppressor genes TSC1 or TSC2, resulting in increased activation of the mTOR pathway. Regarding clinical manifestations, a wide range of phenotypic variability exists, with symptoms constellations that may differ in affected organs (brain, skin, heart, eyes, kidneys, lungs), age of presentation and severity, but usually with great impact in biopsychosocial aspects of health and in quality of life. Main clinical neurological features are epilepsy (frequently, antiepileptic drug-resistant epilepsy), neuropsychiatric disorders, and subependymal giant cell astrocytomas. Recently, many therapeutic strategies have developed, including preventive treatment of epilepsy, new options for treatment of epilepsy as cannabidiol, mTOR inhibitors, ketogenic diet, and a more precise epilepsy surgery. Subependymal giant cell astrocytomas may require surgical procedures or mTOR inhibitors treatment. mTOR inhibitors may also be useful for other comorbidities. To improve quality of life of patients with tuberous sclerosis complex, it is essential to be able to deliver an integrated approach by specialized multidisciplinary teams, coordinated with primary care physicians and health professionals, that include access to treatments, attention of psychosocial aspects, and an adequate health care transition from pediatric to adult care.

El complejo de esclerosis tuberosa es un trastorno genético autosómico dominante multisistémico, causado primariamente por mutaciones en uno de los genes supresores de tumores TSC1 o TSC2, que generan una sobre-activación de la vía mTOR. En relación a las manifestaciones clínicas, existe una gran variabilidad fenotípica, con constelaciones de síntomas que pueden diferir tanto en los órganos afectados (encéfalo, piel, corazón, ojos, riñones, pulmones), como en la edad de presentación y la gravedad, pero que generalmente impactan fuertemente en los aspectos biopsicosociales y en la calidad de vida. Las principales manifestaciones clínicas neurológicas incluyen la epilepsia (con frecuencia, fármaco-resistente), los trastornos neuropsiquiátricos y los síntomas relacionados a los astrocitomas de células gigantes. En los últimos años han surgido nuevos abordajes terapéuticos, incluyendo el tratamiento preventivo de la epilepsia, nuevas opciones de tratamiento para la epilepsia como el uso de cannabidiol, de inhibidores de mTOR, la terapia cetogénica y cirugía de epilepsia. Los astrocitomas de células gigantes pueden requerir tratamientos quirúrgicos o con inhibidores de mTOR. Estos últimos también pueden utilizarse para el tratamiento de otras comorbilidades. Para mejorar la calidad de atención de los pacientes con esclerosis tuberosa, el desafío es poder brindar un abordaje integral por equipos interdisciplinarios especializados, en coordinación con sus médicos de cabecera y los equipos de salud del primer nivel de atención, que incluya el acceso a los tratamientos, la atención de los aspectos psicosociales, y una adecuada transición desde la atención pediátrica a la de adultos.

Concordance and diagnostic yield of stereotactic biopsies for posterior fossa: Technique and experience in a reference hospital.

AIM: Describe our stereotactic brain biopsy (SBB) technique for intra-axial lesions of the posterior fossa, evaluate its effectiveness and safety, and compare them with other series. MATERIAL AND METHODS: Retrospective study in ten consecutive patients, whose variables were age, gender, location of the lesions, clinical, radiological, and histopathological diagnoses, complications, and mortality, for analysis using descriptive statistics and tests of concordance and diagnostic validity. RESULTS: Lesions were pontine in seven cases, and pontomedullary in three occasions, with histopathological diagnoses of four Grade II astrocytomas, two Grade IV astrocytomas, two infectious process, one neuroblastic tumor, and one cavernous malformation, whose frequency differs from the previous reports (χ2 = 0.07). The clinical-radiological concordance was poor (κ = 0.20). The validity of the clinical diagnosis had intermediate values (Sn = 66.7%, Sp = 75%), while radiological studies were more sensitive (Sn = 100%, Sp = 25%). A definitive diagnosis was obtained in all procedures, with no permanent morbidity or mortality because of the surgery. CONCLUSION: The SBB technique for posterior fossa implemented in our hospital shows high diagnostic yield, as well as absolute safety for the patient.

OBJETIVO: Describir nuestra técnica de biopsia cerebral estereotáctica (SBB) para lesiones intraaxiales de fosa posterior, evaluar su eficacia y seguridad y compararlas con otras series. MATERIAL Y MÉTODOS: Estudio retrospectivo en 10 pacientes consecutivos, cuyas variables fueron edad, sexo, localización de las lesiones, diagnósticos clínicos, radiológicos e histopatológicos, complicaciones y mortalidad, para análisis mediante estadística descriptiva y pruebas de concordancia y validez diagnóstica. RESULTADOS: Las lesiones fueron pontinas en 7 casos y pontomedulares en 3 ocasiones, con diagnósticos histopatológicos de 4 astrocitomas grado II, 2 astrocitomas grado IV, 2 procesos infecciosos, 1 tumor neuroblástico y 1 malformación cavernosa, cuya frecuencia difiere de reportes previos (χ2 = 0.07). La concordancia clínico-radiológica fue mala (κ = 0.20). La validez del diagnóstico clínico tuvo valores intermedios (Sn = 66.7%, Sp = 75%), mientras que los estudios radiológicos fueron más sensibles (Sn = 100%, Sp = 25%). Se obtuvo un diagnóstico definitivo en todos los procedimientos, sin morbimortalidad permanente por la cirugía. CONCLUSIÓN: La técnica SBB para fosa posterior implementada en nuestro hospital muestra un alto rendimiento diagnóstico, así como una seguridad absoluta para el paciente.

Pleomorphic Xanthoastrocytoma with Oligodendroglioma-Like Areas with Negative 1p19q Co-Deletion

The most common mixed glioma encountered in routine surgical practice is oligoastrocytoma (OA); however, its is currently considered a vanishing entity. The 2016 classification of the World Health Organization (WHO) discourages the diagnosis of tumors as mixed glioma. The recommendations are that diffuse gliomas, including those withmixed or ambiguous histological features, should be subjected tomolecular testing. Dual-genotype OAs are not yet a distinct entity or variant in the classification. We report a case ofmixed glioma: a pleomorphic xanthoastrocytoma (PXA)mixed with an oligodendroglioma. The immunohistochemistry (IHC) pattern of isocitrate dehydrogenase 1 (IDH1) negativity with retained nuclear expression of the alpha-thalassemia x-linked intellectual disability syndrome (ATRX) protein, and 1p19q co-deletion negativity in both the components enabled its identification as a mixed glioma rather than a collision tumor. To the best of our knowledge, the case herein presented is the fourth case of PXA with oligodendroglioma. Out of the other three reported cases, only one was of a collision tumor with a dual genotype, and the other two showed similar molecular signatures in both components. The present article discusses the histological, immunohistochemical and molecular features of the aforementioned case.

The Resection of a Thalamic Pilocytic Astrocytoma Through the Transchoroidal Fissure, Transcallosal Approach: 2-Dimensional Operative Video.

Surgical resection is the primary treatment of pilocytic astrocytomas and total removal can be curative. However, these lesions occur in critical areas, such as the thalamus, being surrounded by critical life neurovascular structures, which imposes a surgical challenge.1-5 Exhaustive acquisition and meticulous interpretation of preoperative radiological exams; reliable surgical orientation based on profound microneurosurgical anatomic knowledge and judicious discernment of the neuroanatomic distortions on the surface and deep-seated structures inflicted by the neuropathological entity; embracing and comprehensive application of the vast scope of available intraoperative guidance imaging and neurophysiological monitoring; in alliance with the mastered carefully microsurgical technique supported by endoscopic visualization are the keystones to the pursed duet "cure with quality of life" in the treatment of these lesions. We present the case of a 17-yr-old young lady with a progressive motor deficit in her right hemibody for over 2 yr. Her radiological investigation demonstrated a left thalamic lesion displacing the projection fibers (corticospinal tract) within the internal capsule laterally. The patient consented to the surgical procedure. The surgical strategy, intraoperative findings, and microsurgical and endoscopic technique, as well as the postoperative radiological and clinical evaluation are presented. The patient gave her informed consent for the publication of the case.

Radiotherapy in adult low-grade glioma: nationwide trends in treatment and outcomes.

BACKGROUND: Management of WHO grade II gliomas (LGG) can include a combination of observation, surgery, radiotherapy (RT), and chemotherapy; however, optimal management remains unclear in regards to RT. OBJECTIVE: The current study seeks to investigate the usage of RT in LGG and its effect on survival outcomes. METHODS: Patients with diagnosis codes specific for LGG were queried from the National Cancer Database (NCDB) during the years 2004-2016. Kaplan-Meier curves with log-rank testing, univariate and multivariate Cox regression analysis, and comparisons of estimated 3- and 7-year survival were performed to investigate the effect of RT on overall survival. RESULTS: 19,382 patients with LGG were identified with histologically confirmed disease. Kaplan-Meier testing demonstrated RT impacted survival in patients undergoing biopsy or no surgery (p < 0.0001), no chemotherapy (p < 0.0001), and in regimens with early RT (p < 0.0001) and high-dose RT (p < 0.0001). Cox multivariate regression demonstrated RT and age less than 40 (HR 0.93, 95% CI 0.89-0.97, p = 0.001), no chemotherapy (HR 0.82, 95% CI 0.77-0.87, p < 0.001), and astrocytoma histology (HR 0.72, 95% CI 0.66-0.79, p < 0.001) were associated with improved survival. 3-year survival of RT versus non-RT groups showed increased survival rates for age less than 40 years (+ 5.7%, p < 0.0001), no surgery or biopsy (+ 8.1%, p < 0.0001), no chemotherapy (+ 10.3%, p < 0.0001), mixed glioma (+ 6.7%, p < 0.0001), astrocytoma (+ 7.1%, p < 0.0001), and in regimens with early RT (+ 7.6%, p < 0.0001) and high-dose RT (+ 4.7%, p < 0.0001). CONCLUSION: This nationwide analysis of LGG patients found that RT was associated with improved survival outcomes in patients less than 40 years of age, with histology subtypes of astrocytoma and mixed glioma, undergoing biopsy or no surgery, and in regimens with early RT and high-dose RT.

Adult Pilocytic Astrocytomas: A Brazilian Series.

OBJECTIVE: Pilocytic astrocytoma (PA) is rare in adults comprising 5.1% of the primary central nervous system tumors. The aim is to describe the first Brazilian series of adult patients with PA and compare its features with the available literature. METHODS: We retrospectively review all patients 18 years or older with PA from our institution's database from 1991 to 2018. We analyzed information regarding clinical presentation, location, imaging features, extent of resection, adjuvant treatments, and follow-up. RESULTS: Twenty-three patients with PA were analyzed: 60.9% male; median age 26 years. The most frequent symptoms were headache (34.8%) and seizure (26.1%). Temporal and parietal lobes were the most common locations, 21.7% each. All patients underwent a surgical procedure, gross total resection in 40.9%, subtotal resection in 22.7%, and biopsy in 27.3%. Adjuvant treatment with radiotherapy was performed in 2 patients. Only 4 patients had disease progression, 2 after gross total resection and 2 after subtotal resection. They were all alive and without evidence of new progression at the last follow-up (October 2018). Median overall survival was not reached after a median follow-up time of 88.9 months. CONCLUSIONS: This is the first Brazilian series regarding adults with PA, and our patients had a favorable outcome as reported in recent literature reviews. The tumor's prevalence reduces within older patients and supratentorial lesions are more frequent, especially on the temporal lobe. There was no significant relationship between location and progression, although according to the literature the extent of resection remains the most important prognostic factor.

Uso de nimotuzumab en paciente con astrocitoma anaplásico / Nimotuzumab in the treatment of a patient with anaplastic astrocytoma

Introducción: Nimotuzumab es una inmunoglobina de isotipo IgG1, obtenido por tecnología de ADN recombinante. La expectativa de vida de niños con tumores cerebrales recurrentes, refractarios a tratamientos a la cirugía, la radioterapia y la quimioterapia es de un mes aproximadamente. Con este tratamiento la supervivencia alcanza 44,5 meses. Objetivos: Presentar el caso clínico de un paciente con diagnóstico de Astrocitoma anaplásico que recibió tratamiento oncoespecífico concurrente con Nimotuzumab. Presentación de caso: Se realizó la descripción del diagnóstico, tratamiento y evolución de un paciente de 31 años de edad que fue diagnosticado con una neoplasia del sistema nervioso central. (Astrocitoma anaplásico). Recibió la combinación terapéutica de cirugía, radioterapia y anticuerpos monoclonales, lográndose una sobrevida de 39 meses. Conclusiones: La adición del anticuerpo monoclonal al tratamiento estándar de los tumores cerebrales aumentó la sobrevida del paciente, convirtiéndose en una alternativa terapéutica segura, ventajosa y factible como parte del tratamiento convencional en las condiciones asistenciales(AU)

Introduction: Nimotuzumab is an IgG1 isotype immunoglobin, obtained by recombinant DNA technology. Life expectancy is approximately one month in children with recurrent brain tumors, refractory to treatments to surgery, radiotherapy and chemotherapy. Survival reaches 44.5 months when using Nimotuzumab. Objectives: To report the clinical case of a patient diagnosed with anaplastic astrocytoma who received concurrent oncospecific treatment with Nimotuzumab. Case report: This paper describes the diagnosis, treatment and evolution of a 31-year-old male patient with neoplasm of the central nervous system (Anaplastic astrocytoma). He received the therapeutic combination of surgery, radiotherapy and monoclonal antibodies, achieving a survival of 39 months. Conclusions: The adding the monoclonal antibody to the standard treatment of brain tumors increased patient survival, making it a safe, advantageous and feasible therapeutic alternative as part of conventional treatment in healthcare conditions(AU)

Astrocitoma medular de alto grado no infiltrante / Astrocitoma medular não infiltrativo. Apresentação do caso / Non-infiltrating medullary astrocytoma. Case presentation

Se presentó el caso de una paciente femenina de 20 años de edad que acudió al servicio de Neurocirugía por presentar dolor en la región lumbar. Al interrogatorio la paciente refirió tener constipación, retención urinaria y dolor lumbar. En los exámenes complementarios lo más significativo fue la resonancia magnética nuclear que mostró una imagen hiperdensa a nivel de D12-L5. Se realizó laminectomía de D12-L5, durotomía y extracción tumoral, fijación transpedicular con tornillos ubicados en T12-L1-L3-L4 con barras de titanio, se obtuvo un diagnóstico de astrocitoma grado II-III. Al analizar los diferentes aspectos del caso en cuestión, los investigadores concluyen que con la conducta terapéutica tomada con este caso se sientan las bases para un correcto manejo de posteriores pacientes(AU)

The case of a 20-year-old female patient who went to the Neurosurgery department for presenting pain in the lumbar region was presented. At the interrogation, the patient reported having constipation, urinary retention and low back pain. In the complementary tests, the most significant was nuclear magnetic resonance, which showed a hyperdense image at the level of D12-L5. Laminectomy of D12-L5, durotomy and tumor extraction, transpedicular fixation with screws located in T12-L1-L3-L4 with titanium bars was performed, a diagnosis of grade II-III astrocytoma was obtained. When analyzing the different aspects of the case in question, the researchers conclude that with the therapeutic behavior taken with this case, the basis is laid for the correct management of subsequent patients(AU)

Foi apresentado o caso de uma paciente de 20 anos que foi ao departamento de Neurocirurgia por apresentar dor na região lombar. No interrogatório, o paciente relatou constipação, retenção urinária e lombalgia. Nos testes complementares, o mais significativo foi a ressonância magnética nuclear, que mostrou uma imagem hiperdensa ao nível de D12-L5. Foi realizada laminectomia de D12-L5, durotomia e extração de tumores, fixação transpedicular com parafusos localizados em T12-L1-L3-L4 com barras de titânio e foi obtido o diagnóstico de astrocitoma grau II-III. Ao analisar os diferentes aspectos do caso em questão, os pesquisadores concluem que, com o comportamento terapêutico adotado neste caso, é lançada a base para o manejo correto dos pacientes subsequentes)AU=

Spinal pleomorphic xanthoastrocytoma: case report and literature review / Xantoastrocitoma pleomórfico espinal: reporte de caso y revisión de la literatura

El Xantoastrocitoma Pleomórfico Espinal (XAPE) es una neoplasia primaria infrecuente del sistema nervioso central, descrita por primer vez en 1979. De estirpe astrocítica y aunque clasificada como grado II por la OMS, pueden existir variantes anaplásicas o malignas. Con localización usualmente temporal, pocos casos espinales se han descrito a la fecha, debido probablemente a un subdiagnóstico o un subregistro. El pronóstico es generalmente bueno y depende en gran medida del tipo histológico y extensión de la resección quirurgica. Describimos el caso de un hombre de 30 años de edad con dorsalgiay paresia del miembro inferior izquierdo. Se encontró en imágenes de resonancia magnética una lesión aparentemente intradural, extraaxial en los segmentos T8-T9 que se llevó a resección. Se confirmó por histopatología la presencia de un XAPE primario. En la presente publicación realizamos una revisión de la literatura disponible.

Spinal Pleomorphic Xanthoastrocytoma (SPXA) is a rare CNS primary neoplasm, first described in 1979. Although of astrocytic lineage and classified as a grade II neoplasm by the WHO, it may be have anaplastic or malignant variants. Usually located in the temporal lobe, few spinal cases have been described to date, probably due to underdiagnosis and underreporting. It usually has a good prognosis, but it depends on its histological type and extent of surgical resection. In this article, we describe the case of a 30-year old male who complained of low back pain and left lower limb paresis. The MRI showed an apparently intradural, extraaxial lesion at T8-T9 segments. The diagnosis of a primary SPXA was confirmed by histopathological studies. In this article, a review of the available literature is presented.

[Astrocytoma and epilepsy. Clinical case]. / Astrocitoma y epilepsia. Caso clínico.

BACKGROUND: Pilocytic astrocytoma is a rare tumour, usually occurring in paediatric ages, and mainly located in the posterior fossa. It can cause hydrocephalus and intracranial hypertension and, less frequently, seizures, or a focal neurological deficit. The main imaging study by magnetic resonance imaging, which shows a tumour with solid and cystic components without peri-lesional swelling. The election treatment is surgical, and the patient is considered cured if a total resection is accomplished. CLINICAL CASE: The case is presented of 22-year-old female patient with a supratentorial pilocytic astrocytoma and epilepsy. Histopathology reported a low grade glial proliferation, with an extensive fibrillar matrix, small cells without atypia, extensive calcifications and piloid areas consisting of bipolar fusiform cells, and some Rosenthal fibres. There were also spongiotic areas consisting of multipolar cells and associated microcysts. The final report was a pilocytic astrocytoma. CONCLUSIONS: Pilocytic astrocytoma is more frequent in paediatric patients and in the posterior fossa. The case presented is of a young female adult with supratentorial location, making it a special case. The surgery achieved a total resection. The long-term prognosis is good, but it is necessary to perform a follow-up, particularly in adult patients because of a higher risk of recurrence.

Concurrencia de meningioma y astrocitoma: breve reporte de caso / Concurrence of meningioma and astrocytoma: brief case report

Las neoplasias intracraneales múltiples son infrecuentes, representan alrededor del 4 por ciento de todos los tumores cerebrales. Estas lesiones se observan ocasionalmente en pacientes con neurofibromatosis y en tratados con radioterapia. Presentamos el caso de un paciente portador de meningioma y una lesión de estirpe glial manejado en nuestro servicio de neurocirugía.

Multiple intracranial neoplasms are rare, representing about 4 percent of all brain tumors. These lesions are occasionaly observed in patients with neurofibromatosis and in patients treated with radiotherapy. We report the case of a patient with a meningioma and a glial lineage lesion , handled in our service of neurosurgery, University of Cartagena, Colombia.

[Pineal region tumors in children: is gross-total resection necessary? A single-center experience]. / Tumores de región pineal en niños: ¿es la resección total necesaria? Experiencia en un centro de referencia.

BACKGROUND: Survival of children with pineal region tumors has increased significantly in the last decade; these tumors have an insidious outcome associated with endocrine disorders with high morbidity and mortality, especially after gross resection. The objective was to report the survival, outcome, morbidity and mortality according to type of surgery, histology and treatment in children with pineal region tumors. METHODS: This retrospective study included all patients of 17 years or less with diagnosis of pineal region tumor, who went over a period of 10 years to a children's hospital. A histopathological review was made, and the extent of resection was determined. The survival was also estimated. RESULTS: Forty-six patients were included, out of which 36 had complete medical records and adequate pathologic material. Gross resection was performed in 24 (66.6 %), and biopsy in 12 (33.3 %); 23 (88 %) patients died; hydroelectrolytic imbalance was the cause of 14 deaths (60 %) and the other nine (39.1 %) were secondary to tumor progression. Ten-years survivals among patients treated with gross resection and biopsy were 52 and 75 %, respectively (p = 0.7). Endocrine alterations were observed in 13 patients (36.1 %); in 10 of these (76.9 %) the total resection was performed. CONCLUSIONS: Pineal region tumors in children can be treated with diagnostic biopsy, followed by adjuvant treatment consisting of chemotherapy and radiotherapy.

Introducción: la supervivencia de los niños con tumores de la región pineal se ha incrementado en la última década; estos tienen una evolución insidiosa asociada con los desórdenes endocrinológicos y una alta morbilidad y mortalidad, sobre todo después de la resección. El objetivo es reportar la supervivencia, la morbilidad y mortalidad según el tipo de cirugía, la histología y el tratamiento en un grupo de niños con tumores de la región pineal. Métodos: estudio retrospectivo que incluyó a todos los pacientes con diagnóstico de tumor de la región pineal menores de 17 años de edad que acudieron en un periodo de 10 años a un hospital de pediatría. Se realizó una revisión histopatológica, se determinó la extensión de la resección y se estimó la supervivencia. Resultados: se incluyeron 46 pacientes, 36 de los cuales tenían expediente completo y material de patología adecuado. La resección total se realizó en 24 (66.6 %) y biopsia en 12 (33.3 %); fallecieron 23 pacientes (88 %) y fue el desequilibrio hidroelectrolítico la causa de 14 defunciones (60 %) y la progresión tumoral la causa de las 9 defunciones restantes (39.1 %). La sobrevida a 10 años de los pacientes tratados con resección total y biopsia fue del 52 y 75 %, respectivamente, con una p = 0.7; se presentaron alteraciones endocrinológicas en 13 (36.1%) pacientes de los cuales a 10 (76.9 %) se les realizó la resección de la tumoración. Conclusión: los tumores de la región pineal en niños se pueden tratar con biopsia diagnóstica seguida de tratamiento adyuvante con quimioterapia y radioterapia.

Biopsy of Brainstem Gliomas Using Flexible Endoscopes.

OBJECTIVES: To describe our experience and the results obtained in performing transventricular brainstem biopsy with the use of flexible neuroendoscops. METHODS: We identified patients who underwent a neuroendoscopic procedure with brainstem lesion biopsy to obtain histopathologic diagnosis and to treat obstructive hydrocephalus. All patients had follow-up examinations at months 1, 3, 6, and 12 postsurgery and then annually. RESULTS: Seven patients had a transventricular biopsy of the brainstem performed. Of those, five were pediatric patients. The median age was 10 years (range: 3-26 years). Five of them were female and two male. Four patients presented with secondary obstructive hydrocephalus. The main clinical presentations were intracranial hypertension syndrome in four patients, motor neuron disease in four patients, two with decreased state of alertness, two with gait ataxia, and one with Parinaud syndrome. The types of tumors found in the histopathology and their location were one ventral (pons) and one aqueductal anaplastic astrocytoma, two ventral, one aqueductal, and one attached to the floor of the fourth ventricle pilocytic astrocytoma and one ventral low-grade astrocytoma. The route taken to approach the ventral tumors was made through premammillary fenestration. The tumors of the aqueduct and floor of the fourth ventricle were approached transaqueductally. CONCLUSION: The use of flexible endoscops for biopsy of ventral, dorsal (tectum lamina quadrigemina), and diffuse brainstem tumors is a useful, effective, and safe procedure that also allows to treat obstructive hydrocephalus secondary to the tumors.

Intraventricular pleomorphic xanthoastrocytoma: a case report.

Pleomorphic xanthoastrocytoma (PXA), an uncommon glial neoplasm, typically affects adolescents and young adults and accounts for less than 1% of all astrocytic neoplasms. The authors present a case of PXA located entirely in the third ventricle in a 24-year-old male patient. The patient presented with a 6-month history of headaches, progressive decline in cognitive function and profound behavioral disturbances. He was admitted to the hospital with signs of increased intracranial pressure. Magnetic resonance imaging showed a well-enhanced solid tumor, located entirely inside the third ventricle. The tumor was totally removed via a right fronto-pterional trans-lamina terminalis approach and neuropathology report confirmed PXA diagnosis. No further treatment was indicated. To the best of our knowledge, this is the first case report of a solid PXA confined to the third ventricle.

Impacto de la cirugía y la radioterapia en el pronóstico funcional y sobrevida de pacientes con astrocitomas / Impact of surgery and radiotherapy on functional prognosis and survival of patients with astrocytomas

INTRODUCCION: Los astrocitomas son los tumores más frecuentes del SNC y constituyen el 40 a 50 por ciento de todos los tumores cerebrales. La sobrevida depende de varios factores clínicos y de tratamiento, y varía en diferentes estudios internacionales, sin embargo no se conoce la sobrevida real de los pacientes operados de astrocitoma en nuestro medio. El presente estudio tiene como objetivo determinar el impacto de la cirugía y la radioterapia complementaria en la sobrevida y el resultado funcional de pacientes con astrocitomas operados en el Hospital Nacional Guillermo Almenara entre los años 2003 y 2009. MATERIALES y METODOS: Se realizó un estudio retrospectivo, observacional y longitudinal de 124 pacientes operados de astrocitoma en el Hospital Guillermo Almenara entre 2003 y el 2009. Los datos fueron recolectados de historias clínicas, reportes operatorios y entrevistas telefónicas. Se clasificó a los pacientes según el tipo histológico de astrocitoma y se analizaron las variables: Grupo de riesgo pronóstico, tipo de tratamiento y extensión de la cirugía, utilizando para el análisis el programa SPSS 15.0. RESULTADOS: De los 124 pacientes, la mayoría 36.3 por ciento (45/124) tuvieron el diagnóstico de astrocitoma grado IV, seguido por el de grado II 30.6 por ciento (38/124), el grado III 28.2 por ciento (35/124) y sólo el 4.8 por ciento (6/124) grado I. La mayor sobrevida se presentó en el grado I (68 meses), seguido por el grado II (56.7 meses), el grado III (34.8 meses) y el grado IV con 11.3 meses. El resultado funcional fue ligeramente mejor en el grupo de resección total radical (90 por ciento con Karnosfky >70) según tipo de tratamiento, y en el grupo de astrocitomas grado II (85 por ciento con Karnosfky >70) según tipo histológico. Según grupo pronóstico, la sobrevida en los grupos de bajo, mediano y alto riesgo fue 74.8, 46.0 y 31 meses respecto para el astrocitoma grado II, de 46.7, 28.1 y 8.5 meses para los astrocitomas grado III...

INTRODUCTION: Astrocytomas are the most common tumors of the CNS and constitute 40-50 per cent of all brain tumors. Survival depends on several clinical factors and treatment, and prognostic is known in different international studies, however, it does not know the actual survival of astrocytoma patients operated in our area. The present study aims to determine the impact of surgery and adjuvant radiotherapy on survival and functional outcome of patients with astrocytomas operated in the Hospital Guillermo Almenara between 2003 and 2009. MATERIALS AND METHODS: A retrospective, observational, longitudinal study of 124 patients operated astrocytoma in the Guillermo Almenara Hospital between 2003 and 2009 was performed. Data were collected from medical records, operative reports, and telephone interviews. Patients were classified according to the histological type of astrocytoma and variables were analyzed: prognostic risk group, treatment type and extent of surgery, using SPSS 15.0. RESULTS: Of the 124 patients, the majority 36.3 per cent (45/124) were diagnosed with astrocytoma grade IV, followed by astrocytoma grade II 30.6 per cent (38/124), grade III 28.2 per cent (35/124) and only 4.8 per cent (6/124) was grade I. The greatest survival occurred in the grade (68 months), followed by grade II (56.7 months), grade In (34.8 months) and 11.3 months with grade IV. Functional outcome was slightly better in the group of patients with total radical resection (90 per cent with Karnosfky >70) according to type of treatment, and in the group of grade II astrocytomas (85 per cent with Karnosfky >70) according to histological type. According prognostic group, survival in the groups of low, medium and high risk was 74.8, 46.0 and 31 months respectively for astrocytoma grade II, 46.7, 28.1 and 8.5 months for grade III astrocytomas; and 12.3, 12.3 and 6.1 months for grade IV. Regarding the type of treatment, longer survival was obtained in the group of surgery plus...

Low-grade astrocytoma: surgical outcomes in eloquent versus non-eloquent brain areas.

A retrospective study of 81 patients with low-grade astrocytoma (LGA) comparing the efficacy of aggressive versus less aggressive surgery in eloquent and non-eloquent brain areas was conducted. Extent of surgical resection was analyzed to assess overall survival (OS) and progression- free survival (PFS). Degree of tumor resection was classified as gross total resection (GTR), subtotal resection (STR) or biopsy. GTR, STR and biopsy in patients with tumors in non-eloquent areas were performed in 31, 48 and 21% subjects, whereas in patients with tumors in eloquent areas resections were 22.5, 35 and 42.5%. Overall survival was 4.7 and 1.9 years in patients with tumors in non-eloquent brain areas submitted to GTR/STR and biopsy (p=0.013), whereas overall survival among patients with tumors in eloquent area was 4.5 and 2.1 years (p=0.33). Improved outcome for adult patients with LGA is predicted by more aggressive surgery in both eloquent and non-eloquent brain areas.

Low-grade astrocytoma: surgical outcomes in eloquent versus non-eloquent brain areas / Astrocitoma de baixo grau: resultado cirúrgico em área eloquente versus não eloquente

A retrospective study of 81 patients with low-grade astrocytoma (LGA) comparing the efficacy of aggressive versus less aggressive surgery in eloquent and non-eloquent brain areas was conducted. Extent of surgical resection was analyzed to assess overall survival (OS) and progression- free survival (PFS). Degree of tumor resection was classified as gross total resection (GTR), subtotal resection (STR) or biopsy. GTR, STR and biopsy in patients with tumors in non-eloquent areas were performed in 31, 48 and 21% subjects, whereas in patients with tumors in eloquent areas resections were 22.5, 35 and 42.5%. Overall survival was 4.7 and 1.9 years in patients with tumors in non-eloquent brain areas submitted to GTR/STR and biopsy (p=0.013), whereas overall survival among patients with tumors in eloquent area was 4.5 and 2.1 years (p=0.33). Improved outcome for adult patients with LGA is predicted by more aggressive surgery in both eloquent and non-eloquent brain areas.

Foi realizado estudo retrospectivo em 81 pacientes com astrocitoma de baixo grau (LGA) comparando a eficácia da ressecção cirúrgica com cirurgia menos agressiva em relação à área eloquente e não eloquente do cérebro. A extensão da ressecção cirúrgica foi analisada para avaliar a sobrevida geral (OS) e o tempo livre de doença (PFS). O grau da ressecção cirúrgica foi classificado como ressecção total (GTR), subtotal (STR) e biópsia. Nos pacientes com lesão em área não eloquente foram realizadas GTR, STR e biópsia em 31, 48 e 21% dos casos, enquanto, naqueles com lesão em área eloquente, em 22,5, 35 e 42,5%, respectivamente. A sobrevida geral foi de 4,7 e 1,9 anos em pacientes com lesões em área não eloquente submetidos à GTR/STR e biópsia (p=0,013). Nos pacientes com lesão em áreas eloquentes, a sobrevida geral foi de 4,5 e 2,1 anos (p=0,33), respectivamente. A extensão da ressecção é fator preditivo de sobrevida tanto nas lesões em áreas eloquentes quanto nas não eloquentes.

Astrocitoma pilomixoide de fossa posterior em criança: relato de caso / Pilomyxoid astrocytoma of the posterior fossa in children: case report

Descrever um caso de astrocitoma pilomixoide, um tumor do sistema nervoso central raro, descrito e classificado recentemente diante de sintomatologia e achados pouco específicos. Análise e descrição de um caso de astrocitoma pilomixoide submetido a tratamento cirúrgico no Instituto José Frota, Fortaleza, Ceará. Paciente evolui com regressão importante dos sintomas, sem indícios de recidiva tumoral. O diagnóstico e a classificação correta dos tumores cerebrais são de extrema importância clínica, pois modificam de forma impactante o prognóstico, assim como a abordagem terapêutica.

To describe a pilomyxoid astrocytoma case, a tumor of the central nervous system rare recently described and classified in the face of a specific symptomatology and little found. Analysis and description of a case of pilomyxoid astrocytoma underwent surgical treatment at the José Frota Institute in Fortaleza, Ceará. Patient developed a significant decrease of symptoms without evidence of tumor relapse. The diagnosis and the correct classification of cerebral tumors are of extreme clinical importance, because it changes so as impacting the prognosis as well as therapeutic approach.

Spatial orientation deficit in children due to cerebellum astrocytoma pediatric tumor obtained by means of the Attentional Network Test.

Cerebellum astrocytomas are the most typical nervous system tumors in children. Several cognitive deficits have been previously described. These deficits are probably produced by cerebellar connection and gray matter damage. The present study examines attentional deficits in children operated on for cerebellum astrocytomas, using an attentional paradigm with theoretical and clinical bases: the Attentional Network Test (ANT). This test was designed considering the attentional network theory proposed by Posner, and its usefulness has been demonstrated in clinical settings. Children operated on for cerebellar astrocytoma showed a mild attentional deficit in the orientation network.

Psicosis persistente luego de cirugía y radioterapia por tumor cerebral: reporte de un caso / Persistent psychosis after brain tumor surgery and radiotherapy: a case report

Se presenta el caso de una mujer de 31 años que fue diagnosticada de astrocitoma frontal hace 10 años. Luego de la resección quirúrgica del tumor y del inicio de la radioterapia, la paciente comenzó a presentar síntomas psicóticos, para los cuales ha sido necesario utilizar diferentes medicamentos antipsicóticos en forma sucesiva y por tiempo prolongado, debido a la escasa respuesta inicial y a la recurrencia de los síntomas.

We present the case of a 31-year-old woman who was diagnosed of a frontal astrocytoma 10 years ago. After surgical resection of the tumor and radiotherapy was started, the patient began to present psychotic symptoms, for which different successive antipsychotic drugs were needed due to initial poor response and recurrence of symptoms.