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1.
Rev. argent. radiol ; 85(1): 3-10, ene. 2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1155707

ABSTRACT

Resumen Objetivo: Analizar características por resonancia magnética (RM) de gliomas IDH-mutados (grado II y III) en base a parámetros cualitativos, a fin de valorar el rendimiento del signo del mismatch T2-FLAIR y otras características morfológicas de los tumores, en predecir el estado del 1p/19q y su reproducibilidad interobservador. Métodos Estudio retrospectivo, descriptivo y analítico sobre una cohorte de 53 gliomas IDH-mutados (grado II y III) y molecularmente definidos respecto al 1p/19q, seleccionados a partir de la base de datos de la institución, durante el periodo 2014- 2019. Dos neuroradiólogos evaluaron características imagenológicas de forma independiente y enmascarada al diagnóstico: mismatch T2-FLAIR, localización tumoral, bordes, señal, infiltración cortical e inhomogeneidad en T2. Los casos discordantes fueron evaluados por un tercer neuroradiólogo de mayor experiencia. Resultados: Treinta de 53 (56,6%) gliomas fueron no codelecionados, y 23/53 (43,4%) codelecionados. El signo del mismatch T2-FLAIR fue positivo en 16/53 (30,18%) pacientes, 15/16 (93,75%) no codelecionados y 1/16 (6,25%) codelecionado (Exacto de Fisher p = <,0001). Los dos evaluadores demostraron una concordancia interobservador casi perfecta para ese signo, κ =,907 (95% CI, 0,781 a 1,0). La especificidad y el valor predictivo positivo del signo para predecir la ausencia de la codeleción fue de un 95,7% y un 93,8% respectivamente. Discusión: La reciente actualización en la clasificación de los gliomas los clasifica acorde a su perfil molecular. En los últimos años, varios investigadores han estudiado características morfológicas por RM de los tumores con la intención de predecir las características moleculares de los mismos. Conclusión: En nuestra población, el signo del mismatch T2-FLAIR es el único biomarcador radiológico que muestra asociación estadísticamente significativa en predecir la ausencia de codeleción en los gliomas IDH-mutados (grado II y III), con una alta especificidad y un alto valor predictivo positivo.


Abstract Objective: To analyze magnetic resonance (MR) characteristics of IDH-mutated gliomas (grades II/III) utilizing qualitative parameters with the goal of assessing the performance of the T2-FLAIR mismatch sign and other morphological characteristics of tumors in predicting the 1p/19q co-deletion status as well as inter-observer reproducibility. Methods: Retrospective and descriptive study analyzing a cohort of 53 IDH-mutated lower-grade (grades II/III) gliomas with known 1p/19q co-deletion status. Patients meeting selection criteria for this study were taken from our institutional data from 2014-2019. Two neuroradiologists assessed the following imaging characteristics independently, and blinded from the diagnosis: T2-FLAIR mismatch, tumor location, borders, signal characteristics, cortical infiltration and T2* inhomogeneity. In the event of discordant interpretations, a third senior neuroradiologist also evaluated the case. Results: 23 of the 53 (43.4%) gliomas demonstrated 1p/19q co-deletion and 30 of 53 (56.6%) did not. T2-FLAIR mismatch was positive in 16 of 53 cases (30.2%) with 15 of 16 (93.8%) demonstrating no co-deletion and 1/16 (6.25%) with co-deletion (Fisher's exact p = < .0001). The two readers showed an almost perfect interreader agreement for this sign κ = 0.907 (95% CI, 0.781 to 1.0). Specificity and positive predictive value of the sign to predict the absence of co-deletion was 95.7% and 93.8% respectively. Discussion: The recent update in classification of lower-grade gliomas segregates gliomas according to molecular profile. In the recent past, many researchers have studied MR morphologic characteristics of these tumors with the intention of predicting molecular features of said tumors Conclusion: In our patient population, T2-FLAIR mismatch sign is the only radiologic biomarker that shows statistically significant association with the absence of 1p/19q co-deletion in lower-grade gliomas, with high specificity and positive predictive value.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Young Adult , Brain Neoplasms/diagnostic imaging , Biomarkers , Glioma/diagnostic imaging , Oligodendroglioma/diagnostic imaging , Astrocytoma/diagnostic imaging , Magnetic Resonance Spectroscopy , Epidemiology, Descriptive , Retrospective Studies , Glioma/classification
2.
Rev. bras. oftalmol ; 79(4): 276-277, July-Aug. 2020. graf
Article in English | LILACS | ID: biblio-1137971

ABSTRACT

Abstract Cerebellar astrocytoma (low-grade glioma) is the most frequent tumor of the Central Nervous System in pediatric age, corresponding to 10-20% of brain tumors, having its maximum incidence at 5 years. Brain tumors are the second cause of death at this age, behind leukemias. Its most frequent clinic is headache with vomiting which can worsen in the morning and awaken the patient at night. The most frequent ophthalmological clinic is papilledema and involvement of the cranial nerve VI. In our case we present an atypical presentation (cranial IV), in which a quick derivation favored a better prognosis.


Resumo O astrocitoma cerebelar (glioma de baixo grau) é o tumor mais frequente do Sistema Nervoso Central em idade pediátrica, correspondendo a 10-20% dos tumores cerebrais, tendo sua incidência máxima em 5 anos. Os tumores cerebrais são a segunda causa de morte nesta idade, atrás das leucemias. Sua clínica mais frequente é a cefaleia com vômitos que podem piorar pela manhã e despertar o paciente à noite. A clínica oftalmológica mais frequente é o papiledema e o envolvimento do nervo craniano VI. Em nosso caso apresentamos uma apresentação atípica (IV craniana), em que uma derivação rápida favoreceu um melhor prognóstico.


Subject(s)
Humans , Child, Preschool , Astrocytoma/diagnostic imaging , Brain Neoplasms/diagnostic imaging , Papilledema/physiopathology , Glioma/diagnostic imaging , Headache/physiopathology , Tomography, X-Ray Computed/methods
3.
Rev. cuba. med ; 58(4): e507, oct.-dic. 2019. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1139034

ABSTRACT

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)


Subject(s)
Humans , Male , Adult , Astrocytoma/diagnosis , Astrocytoma/therapy , Antibodies, Monoclonal, Humanized/therapeutic use , Survival Analysis
4.
Rev. Hosp. Ital. B. Aires (2004) ; 39(4): 115-127, dic. 2019. ilus, tab
Article in Spanish | LILACS | ID: biblio-1099709

ABSTRACT

La neurofibromatosis (NF) comprende un grupo de enfermedades genéticas de herencia autosómica dominante, que se clasifican de la siguiente manera: neurofibromatosis tipo 1 (NF1), neurofibromatosis tipo 2 (NF2) y schwannomatosis (también conocida como neurofibromatosis tipo 3). Esta última es una enfermedad muy infrecuente, con una prevalencia aproximada de 1/126 000 personas, por lo que solo profundizaremos las dos primeras. La NF1, también conocida como la enfermedad de Von Recklinghausen, es la más frecuente de las tres y afecta principalmente la piel y el sistema nervioso periférico. Se caracteriza por la presencia de máculas "café con leche", pecas axilares o inguinales, nódulos de Lisch (hamartomas en el iris) y neurofibromas (tumores de la vaina de nervios periféricos). Otras manifestaciones menos frecuentes, aunque de mayor gravedad, incluyen gliomas del nervio óptico, meningiomas, neurofibromas malignos, escoliosis y displasia de la tibia. Su diagnóstico se suele realizar al nacimiento o durante los primeros años de vida, y se estima que un 50% de quienes la padecen presenta dificultades cognitivas. No hay datos concluyentes sobre la mortalidad en los pacientes con NF1, aunque se sabe que la expectativa de vida es menor que en la población general. La NF2 tiene una prevalencia considerablemente menor que la NF1 y su inicio es más tardío, afectando principalmente a adultos jóvenes. La presentación clínica típica se caracteriza por acúfenos, hipoacusia y ataxia en contexto de la presencia de schwannomas vestibulares bilaterales. Otros hallazgos menos frecuentes incluyen schwannomas de nervios periféricos, meningiomas, ependimomas o astrocitomas. La esperanza de vida es de unos 36 años, con una supervivencia media desde el momento del diagnóstico de 15 años. (AU)


Neurofibromatosis (NF) includes a group of genetic diseases with an autosomal-dominant inheritance pattern, and they are classified as follows: Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and Schwannomatosis (also known as neurofibromatosis type 3). This last one is a very rare disease, with an approximate prevalence of 1/126000, so we will only deepen in the first two. NF1, also known as von Recklinghausen disease, is the most frequent, and mainly affects the skin and peripheral nervous system. Its typical manifestations are the presence of café-au-lait macules, axillary or inguinal freckles, Lisch nodules (hamartomas in the iris) and neurofibromas (peripheral nerve sheath tumors). Less frequent manifestations, although more serious, include optic nerve gliomas, meningiomas, malignant neurofibromas, scoliosis and tibial dysplasia. The diagnosis is usually made at birth or during the first years of life, and approximately 50% of patients present cognitive difficulties. There is no conclusive data on mortality in patients with NF1, although it is known that life expectancy is lower than in general population. NF2 has a considerably lower prevalence than NF1, and its onset is later in life, mainly affecting young adults. Its typical clinical presentation is characterized by tinnitus, hearing loss and ataxia in the context in the presence of bilateral vestibular schwannomas. Less frequent findings include peripheral nerve schwannomas, meningiomas, ependymomas or astrocytomas. Life expectancy is about 36 years old, with a median survival from the moment of diagnosis of 15 years. (AU)


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Adult , Young Adult , Neurofibromatosis 2/etiology , Neurofibromatosis 1/etiology , Neurofibromatoses/classification , Astrocytoma/physiopathology , Ataxia , Scoliosis/physiopathology , Tibia/abnormalities , Tinnitus , Bone Diseases, Developmental/physiopathology , Neuroma, Acoustic/complications , Life Expectancy , Neurofibromatosis 2/epidemiology , Neurofibromatosis 1/physiopathology , Neurofibromatosis 1/mortality , Neurofibromatosis 1/epidemiology , Neurofibromatoses/diagnosis , Optic Nerve Glioma/physiopathology , Ependymoma/physiopathology , Hearing Loss , Iris Diseases/physiopathology , Melanosis/physiopathology , Meningioma/physiopathology , Neurilemmoma/etiology , Neurilemmoma/physiopathology , Neurofibroma/physiopathology , Neurofibroma/pathology
5.
Article in English | WPRIM | ID: wpr-739666

ABSTRACT

Supratentorial extraventricular anaplastic ependymoma (SEAE) in adults is a relatively rare intracranial tumor. Because of the very low prevalence, only a few cases have been reported. According to a recent study, SEAE is associated with a poor prognosis and there is no definite consensus on optimal treatment. We report a case of an adult SEAE patient who had no recurrence until seven years after a gross total resection (GTR) followed by conventional radiotherapy. A 42-year-old male had a persistent mild headache, left facial palsy, dysarthria, and left hemiparesis. Preoperative neuroimaging revealed an anaplastic astrocytoma or supratentorial ependymoma in the right frontal lobe. A GTR was performed, followed by adjuvant radiotherapy. Histologic and immunohistochemical results revealed anaplastic ependymoma. After seven years of initial therapy, a regular follow-up MRI showed a 3-cm-sized partially cystic mass in the same area as the initial tumor. The patient underwent a craniotomy, and a GTR was performed. Histopathologic examination revealed recurrence of the SEAE. External radiotherapy was performed. The patient has been stable without any disease progression or complications for 12 months since the surgery for recurrent SEAE.


Subject(s)
Adult , Astrocytoma , Consensus , Craniotomy , Disease Progression , Dysarthria , Ependymoma , Facial Paralysis , Follow-Up Studies , Frontal Lobe , Headache , Humans , Magnetic Resonance Imaging , Male , Neuroimaging , Paresis , Prevalence , Prognosis , Radiotherapy , Radiotherapy, Adjuvant , Recurrence , Supratentorial Neoplasms
6.
Article in English | WPRIM | ID: wpr-766040

ABSTRACT

BACKGROUND: The 2016 World Health Organization (WHO) classification of central nervous system (CNS) tumors has been modified to incorporate the IDH mutation and 1p/19q co-deletion in the diagnosis of diffuse gliomas. In this study, we aimed to evaluate the feasibility and prognostic significance of the revised 2016 WHO classification of CNS tumors in Mongolian patients with diffuse gliomas. METHODS: A total of 124 cases of diffuse gliomas were collected, and tissue microarray blocks were made. IDH1 mutation was tested using immunohistochemistry, and 1p/19q co-deletion status was examined using fluorescence in situ hybridization analysis. RESULTS: According to the 2016 WHO classification, 124 cases of diffuse brain glioma were reclassified as follows: 10 oligodendroglioma, IDHmut and 1p/19q co-deleted; three anaplastic oligodendroglioma, IDHmut and 1p/19q co-deleted; 35 diffuse astrocytoma, IDHmut, 11 diffuse astrocytoma, IDHwt, not otherwise specified (NOS); 22 anaplastic astrocytoma, IDHmut, eight anaplastic astrocytoma, IDHwt, NOS; and 35 glioblastoma, IDHwt, NOS, respectively. The 2016 WHO classification presented better prognostic value for overall survival in patients with grade II tumors than traditional histological classification. Among patients with grade II tumors, those with oligodendroglioma IDHmut and 1p/19q co-deleted and diffuse astrocytoma IDHmut showed significantly higher survival than those with diffuse astrocytoma IDHwt, NOS (p<.01). CONCLUSIONS: Mongolian diffuse gliomas could be reclassified according to the new 2016 WHO classification. Reclassification revealed substantial changes in diagnosis of both oligodendroglial and astrocytic entities. We have confirmed that the revised 2016 WHO CNS tumor classification has prognostic significance in Mongolian patients with diffuse gliomas, especially those with grade II tumors.


Subject(s)
Astrocytoma , Brain , Central Nervous System , Chromosome Deletion , Classification , Diagnosis , Fluorescence , Glioblastoma , Glioma , Global Health , Humans , Immunohistochemistry , In Situ Hybridization , Isocitrate Dehydrogenase , Nervous System Neoplasms , Nervous System , Oligodendroglioma , World Health Organization
7.
Article in English | WPRIM | ID: wpr-765349

ABSTRACT

Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.


Subject(s)
Age of Onset , Astrocytoma , Biology , Brain , Brain Neoplasms , Classification , Epilepsy , Ganglioglioma , Glioma , Humans , Molecular Targeted Therapy , Neoplasms, Neuroepithelial , Seizures , Temporal Lobe
8.
Article in English | WPRIM | ID: wpr-763112

ABSTRACT

BACKGROUND: There was no practical guideline for the management of patients with central nervous system tumor in Korea in the past. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, developed the guideline for glioblastoma successfully and published it in Brain Tumor Research and Treatment, the official journal of KSNO, in April 2019. Recently, the KSNO guideline for World Health Organization (WHO) grade III cerebral glioma in adults has been established. METHODS: The Working Group was composed of 35 multidisciplinary medical experts in Korea. References were identified by searches in PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL databases using specific and sensitive keywords as well as combinations of keywords. Scope of the disease was confined to cerebral anaplastic astrocytoma and oligodendroglioma in adults. RESULTS: Whenever radiological feature suggests high grade glioma, maximal safe resection if feasible is globally recommended. After molecular and histological examinations, patients with anaplastic astrocytoma, isocitrate dehydrogenase (IDH)-mutant should be primary treated by standard brain radiotherapy and adjuvant temozolomide chemotherapy whereas those with anaplastic astrocytoma, NOS, and anaplastic astrocytoma, IDH-wildtype should be treated following the protocol for glioblastomas. In terms of anaplastic oligodendroglioma, IDH-mutant and 1p19q-codeletion, and anaplastic oligodendroglioma, NOS should be primary treated by standard brain radiotherapy and neoadjuvant or adjuvant PCV (procarbazine, lomustine, and vincristine) combination chemotherapy. CONCLUSION: The KSNO's guideline recommends that WHO grade III cerebral glioma of adults should be treated by maximal safe resection if feasible, followed by radiotherapy and/or chemotherapy according to molecular and histological features of tumors.


Subject(s)
Adult , Astrocytoma , Brain , Brain Neoplasms , Central Nervous System , Drug Therapy , Drug Therapy, Combination , Glioblastoma , Glioma , Humans , Isocitrate Dehydrogenase , Korea , Lomustine , Oligodendroglioma , Radiotherapy , World Health Organization
9.
Article in English | WPRIM | ID: wpr-763111

ABSTRACT

BACKGROUND: There was no practical guideline for the management of patients with central nervous system tumor in Korea for many years. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has developed the guideline for glioblastoma. Subsequently, the KSNO guideline for World Health Organization (WHO) grade II cerebral glioma in adults is established. METHODS: The Working Group was composed of 35 multidisciplinary medical experts in Korea. References were identified by searching PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL databases using specific and sensitive keywords as well as combinations of keywords regarding diffuse astrocytoma and oligodendroglioma of brain in adults. RESULTS: Whenever radiological feature suggests lower grade glioma, the maximal safe resection if feasible is recommended globally. After molecular and histological examinations, patients with diffuse astrocytoma, isocitrate dehydrogenase (IDH)-wildtype without molecular feature of glioblastoma should be primarily treated by standard brain radiotherapy and adjuvant temozolomide chemotherapy (Level III) while those with molecular feature of glioblastoma should be treated following the protocol for glioblastomas. In terms of patients with diffuse astrocytoma, IDH-mutant and oligodendroglioma (IDH-mutant and 1p19q codeletion), standard brain radiotherapy and adjuvant PCV (procarbazine+lomustine+vincristine) combination chemotherapy should be considered primarily for the high-risk group while observation with regular follow up should be considered for the low-risk group. CONCLUSION: The KSNO's guideline recommends that WHO grade II gliomas should be treated by maximal safe resection, if feasible, followed by radiotherapy and/or chemotherapy according to molecular and histological features of tumors and clinical characteristics of patients.


Subject(s)
Adult , Astrocytoma , Brain , Central Nervous System , Drug Therapy , Drug Therapy, Combination , Follow-Up Studies , Glioblastoma , Glioma , Humans , Isocitrate Dehydrogenase , Korea , Oligodendroglioma , Radiotherapy , World Health Organization
10.
Article in English | WPRIM | ID: wpr-763109

ABSTRACT

BACKGROUND: Pilocytic astrocytoma (PA) is a brain tumor that is relatively more common in children and young adults. METHODS: We retrospectively reviewed the medical records of patients with PA treated at a single center between 1988 and 2018. RESULTS: We included 31 subjects with PA. The median age at diagnosis was 13.4 years, and the median follow-up duration was 9.9 years. The total PA group had a 10-year disease-specific survival (DSS) rate of 92.6% [95% confidence interval (CI), 82.6–100] and 10-year progression-free survival (PFS) rate of 52.8% (95% CI, 32.0–73.6). In patients aged <20 years, tumors were more likely to be located in sites in which gross total tumor resection (GTR) was impossible. No statistically significant difference in 10-year DSS was found between the GTR (100%) and non-GTR (89.7%; 95% CI, 76.2–100; p=0.374) groups. However, a statistically significant difference in 10-year PFS was found between the GTR (100%) and non-GTR groups (30.7%; 95% CI, 8.6–52.8; p=0.012). In the non-GTR group, no statistically significant difference in 10-year DSS was found between the patients who received immediate additional chemotherapy and/or radiotherapy (Add-Tx group, 92.9%; 95% CI, 79.4–100) and the non-Add-Tx group (83.3%; 95% CI, 53.5–100; p=0.577). No statistically significant difference in 10-year PFS was found between the Add-Tx group (28.9%; 95% CI, 1.7–56.1) and non-Add-Tx group (33.3%; 95% CI, 0–70.9; p=0.706). CONCLUSION: The PFS of the patients with PA in our study depended only on the degree of surgical excision associated with tumor location. This study is limited by its small number of patients and retrospective nature. A multicenter and prospective study is necessary to confirm these findings.


Subject(s)
Adolescent , Astrocytoma , Brain Neoplasms , Child , Diagnosis , Disease-Free Survival , Drug Therapy , Follow-Up Studies , Glioma , Humans , Medical Records , Prognosis , Prospective Studies , Radiotherapy , Retrospective Studies , Survivors , Young Adult
11.
Article in English | WPRIM | ID: wpr-763108

ABSTRACT

BACKGROUND: Numerous studies reported a usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-guided surgery (FGS) in high grade gliomas. However, fluorescence patterns and intensities are variable among gliomas. In this study, we report our extensive experience with FGS in various gliomas, focusing on epidemiological data of fluorescence patterns. METHODS: A total of 827 histologically proven glioma patients out of 900 brain tumor patients who had undergone FGS using 5-ALA during the period of 8.5 years between July 2010 and January 2019 were analyzed. Indications of FGS in glioma surgery are evidence for possible high-grade foci in putative gliomas in preoperative MRI. RESULTS: Among the 827 gliomas, the number of cases corresponding to 2016 World Health Organization (WHO) grade IV, III, II, and I are 528 (58.7%), 193 (21.4%), 87 (9.7%) and 19 (2.1%), respectively. In terms of fluorescence rate, grade IV gliomas showed positive fluorescence in 95.4% of cases including strong intensity in 85.6%. Grade III gliomas showed fluorescence in about half of cases (55.0%), but 45.0% of the cases showed no fluorescence at all. Anaplastic oligodendroglioma had a higher positive rate (63.9%) than anaplastic astrocytoma (46.2%). Both grade II and I gliomas still showed positive fluorescence in about one-fourth of cases (24.1% and 26.3% respectively). Among them ependymoma and pilocytic astrocytoma were fluorescence-prone tumors. CONCLUSION: This epidemiological data of 5-ALA fluorescence in various grades of glioma provides a basic reference to the clinical application of FGS with 5-ALA in glioma surgery.


Subject(s)
Astrocytoma , Brain Neoplasms , Ependymoma , Fluorescence , Glioblastoma , Glioma , Humans , Magnetic Resonance Imaging , Oligodendroglioma , World Health Organization
12.
Fisioter. Mov. (Online) ; 32: e003219, 2019. tab, graf
Article in English | LILACS | ID: biblio-1012116

ABSTRACT

Abstract Introduction: Constraint-induced movement therapy (CIMT) is a very modern method used in rehabilitation to treat individuals with functional impairment of the upper limbs. Objective: To apply the CIMT method and evaluate its effects in a patient with ataxia after removal of a cerebellar tumor. Method: This is a longitudinal interventional study of the case report type. The participant complained of tremors and difficulty writing and typing with the right hand as a result of the cerebellar tumor's removal. She was subjected to CIMT daily in three-hour sessions for 10 days. The participant was evaluated with the Motor Activity Log (MAL) and the Wolf Motor Function Test (WMFT). Results: There was an improvement in the quantity, quality, speed, accuracy and strength with which the participant performed the tasks according to the MAL and WMFT scales. Improvement in writing was also identified and the report of better use of the member for daily tasks demonstrated the patient's satisfaction after the end of therapy. Conclusion: This case report demonstrated the benefits of the application of the CIMT method in a patient with ataxia as a consequence of a rare cerebellar tumor, improving task execution time, strength and functional improvement of the affected upper limb.


Resumo Introdução: A terapia por contensão induzida (TCI) é um método atual de reabilitação com intuito de tratar indivíduos com perdas funcionais nos membros superiores. Objetivo: Aplicar o método TCI e avaliar seus efeitos em uma paciente com ataxia após a remoção de tumor cerebelar. Método: Trata-se de um estudo longitudinal de caráter intervencionista do tipo relato de caso. A participante queixava-se de tremor e dificuldade de escrever e digitar com a mão direita em consequência da retirada de um tumor cerebelar. Foi tratada segundo método de TCI por três horas diárias durante 10 dias e avaliada pela Motor Activity Log (MAL) e Wolf Motor Activity Test (WMFT). Resultados: Houve melhora na quantidade, qualidade, velocidade, exatidão e força com que a participante executava as tarefas de acordo com as escalas MAL e WMFT. A melhora na escrita também foi identificada e o relato de melhor uso do membro para tarefas cotidianas demostrou satisfação da paciente após o final da terapia. Conclusão: Esse relato de caso demostrou os benefícios da aplicação do método TCI em uma paciente com ataxia como sequela de um tumor cerebelar raro, o que proporcionou melhora no tempo de execução de tarefas força e ganho funcional no membro afetado.


Resumen Introducción: La terapia por contención inducida (TCI) es un método actual de rehabilitación com el propósito de tratar individuos con pérdidas funcionales en los miembros superiores. Objetivo: Aplicar el método TCI y evaluar sus efectos en una paciente con ataxia después de la remoción del tumor cerebeloso. Método: Se trata de una investigación longitudinal de carácter intervencionista del tipo informe de caso. Una participante se quejaba de temblor y dificultad para escribir con la mano derecha, como consecuencia de la remoción de un tumor cerebeloso. Se la trató según el método de terapia inducida por tres horas diarias durante 10 días e, después de la evaluación del registro de actividades motoras Motor Activity Log (MAL) y Wolf Motor Activity Test (WMFT). Resultados: Se ha mejorado la cantidad, calidad, velocidad, exactitud y en la fuerza con que la participante desarrollaba sus tareas de acuerdo con las escalas MAL y WMFT. También se identificó una mejora en la escritura, además del relato de mejor uso del miembro para tareas de la vida cotidiana, lo que demuestra la satisfacción de la paciente después del fin de la terapia. Conclusión: Este relato de caso demuestra los beneficios de la aplicación del método TCI en una paciente con ataxia como secuela de un tumor cerebeloso raro, lo que proporcionó una mejora en el tiempo de ejecución de tareas, fuerza y proporciono provechos funcionales en el miembro afectado.


Subject(s)
Astrocytoma , Ataxia , Neurological Rehabilitation , Upper Extremity
13.
Rev. inf. cient ; 98(5): 640-647, 2019. ilus
Article in Spanish | LILACS | ID: biblio-1024875

ABSTRACT

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=


Subject(s)
Female , Astrocytoma/surgery , Astrocytoma/diagnosis , Laminectomy
14.
Article in English | WPRIM | ID: wpr-788777

ABSTRACT

Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.


Subject(s)
Age of Onset , Astrocytoma , Biology , Brain , Brain Neoplasms , Classification , Epilepsy , Ganglioglioma , Glioma , Humans , Molecular Targeted Therapy , Neoplasms, Neuroepithelial , Seizures , Temporal Lobe
15.
Rev. Assoc. Med. Bras. (1992) ; 64(12): 1129-1133, Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-976809

ABSTRACT

SUMMARY OBJECTIVE: This study aims to compare estrogen receptor expression between low and high-grade astrocytomas. METHOD: A study using paraffin blocks of glial tumors from the Anatomy Pathology archives of São Marcos Hospital was carried out and began after approval by the Review Board of the Federal University of Piaui. Specimens were histochemically marked with an anti-ER alpha antibody. Brown-stained nuclei were considered positive, regardless of reaction intensity. Data were statistically analyzed using the Mann-Whitney test and Spearman's correlation. Statistical significance was established at p<0.05. RESULTS: The mean percentage of nuclei stained with anti-ER alpha in low-and high-grade astrocytomas was 0.04 and zero, respectively, while Spearman's correlation showed a strong negative association between low and high-grade tumors (p<0.001) and (r= −0.67), respectively. CONCLUSION: In the current study, estrogen receptor expression was positive only in low-grade astrocytomas and nil in high-grade astrocytomas, showing that ER expression declines with the grade of tumor malignancy.


RESUMO OBJETIVO: O objetivo deste estudo é comparar a expressão do receptor de estrogênio entre astrocitomas de baixo e alto grau. MÉTODO: Foi realizado um estudo usando blocos de parafina de tumores gliais dos arquivos de Anatomia Patológica do Hospital São Marcos e iniciado após aprovação pelo Comitê de Ética da Universidade Federal do Piauí. Os espécimes foram marcados histoquimicamente com anticorpo anti-ER alpha. Os núcleos corados em marrom foram considerados positivos, independentemente da intensidade da reação. Os dados foram analisados estatisticamente utilizando o teste de Mann-Whitney e a correlação de Spearman. A significância estatística foi estabelecida em p<0,05. RESULTADOS: A porcentagem média de núcleos corados com anti-ER alfa em astrocitomas de baixo e alto grau foi de 0,04 e zero, respectivamente, enquanto a correlação de Spearman mostrou uma forte correlação negativa entre tumores de baixa e alta qualidade (p<0,001) e (r=-0,67), respectivamente. CONCLUSÕES: No presente estudo, a expressão do receptor de estrogênio foi positiva apenas em astrocitomas de baixo grau e nula em astrocitomas de alto grau, mostrando que a expressão de ER diminui com o grau de malignidade tumoral.


Subject(s)
Humans , Astrocytoma/metabolism , Brain Neoplasms/metabolism , Receptors, Estrogen/metabolism , Biomarkers, Tumor/metabolism , Gene Expression Regulation, Neoplastic , Astrocytoma/pathology , Brain Neoplasms/pathology , Immunohistochemistry , Neoplasm Grading
16.
Rev. colomb. cancerol ; 22(4): 157-161, oct.-dic. 2018. graf
Article in Spanish | LILACS | ID: biblio-985458

ABSTRACT

Resumen El astrocitoma con diferenciación gemistocítica es una variante histológica de los astrocitomas difusos que se caracteriza por presentar tendencia a una rápida progresión hacia la malignidad, y a pesar de ser clasificado según la Organización Mundial de la Salud como un glioma grado II, presenta pobre pronóstico a corto plazo. La presentación clínica más común de este tipo de tumores son los síndromes convulsivos y los déficits focales propios de la localización, mientras que el síndrome de hipertensión endocraneana es poco común. Como primera manifestación se encuentra irritación meníngea más aún en ausencia de infección del sistema nervioso central. Se reporta el caso de un paciente que ingresó a servicio de urgencias con irritación meníngea, a quien finalmente se le diagnostica astrocitoma gemistocitico intraventricular.


Abstract An astrocytoma with gemistocytic differentiation is a histological variant of diffuse astrocytomas, characterised by a tendency towards a rapid progression to malignancy. Despite being classified as a grade II glioma according to the World Health Organisation, it has poor short-term prognosis. The most common clinical presentation of these types of tumours are, convulsive syndrome, focal deficits secondary to the location of the tumour, and / or intracranial hypertension syndrome. It is uncommon to find meningeal irritation as a first manifestation, and even more, in the absence of infection of the central nervous system. The case is presented on a patient, who was admitted to the Emergency Department with signs of meningeal irritation. A diagnosis of intraventricular gemistocytic astrocytoma was finally made.


Subject(s)
Humans , Astrocytoma , Intracranial Hypertension , Meningeal Neoplasms , Prognosis
17.
Arch. argent. pediatr ; 116(5): 667-670, oct. 2018. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-973670

ABSTRACT

El síndrome diencefálico es una causa infrecuente de desnutrición. Se produce por la disfunción del hipotálamo y está asociado a tumores del encéfalo. Los pacientes presentan una grave y progresiva pérdida de peso, aunque el apetito y la ingesta calórica son, por lo general, adecuados. Característicamente, los síntomas neurológicos son tardíos, lo que retrasa la sospecha diagnóstica. Se presenta a un paciente de 2 años y 6 meses de edad con desnutrición crónica grado II, derivado con diagnóstico presuntivo de enfermedad celíaca con mala adherencia y fracaso del tratamiento. Durante la internación, se arribó al diagnóstico de síndrome diencefálico secundario a un astrocitoma pilocítico grado I.


Diencephalic syndrome is an infrequent cause of malnutrition. It is produced by a malfunctioning hypothalamus, and it is related to encephalic tumors. Patients present a serious and progressive weight loss although the appetite and calorie intake are, usually, adequate. Neurological symptoms typically have a late appearance, delaying diagnostic suspicion. We present a patient aged 2 years and a half with grade II chronic malnutrition, referred with presumptive diagnosis of celiac disease, with poor adherence and treatment failure. During hospitalization, diagnosis of diencephalic syndrome secondary to grade I pilocytic astrocytoma was reached.


Subject(s)
Humans , Male , Child, Preschool , Astrocytoma/diagnosis , Child Nutrition Disorders/diagnosis , Celiac Disease/diagnosis , Hypothalamic Diseases/diagnosis , Astrocytoma/complications , Chronic Disease , Hypothalamic Diseases/etiology
18.
Arq. bras. neurocir ; 37(1): 54-57, 13/04/2018.
Article in English | LILACS | ID: biblio-911369

ABSTRACT

The pilomyxoid astrocytoma (PMA) is a rare glioma recently described as a separate entity, which is generally located on the hypothalamic area. The PMA was previously described as pilocytic astrocytoma (PA) due to similarities shared between them. Recent studies provided a deeper understanding of PMA, setting it as a separate entity, though PMA is still considered by many authors a variant of PA. The PMA is considered to be more aggressive than PA; however, further studies are necessary for a better comprehension of its behavior and, hence, for neurosurgeons and neurologists to get to a consensus about its management. This study presents a 16-year-old female patient who looked for medical assistance complaining of headaches of over 6 months and vomiting for 2 weeks prior to the visit to the doctor. She presented no other symptoms. The physical examination displayed only bilateral papilledema. The magnetic resonance imaging (MRI) scans showed an intraventricular and thalamic lesion composed of solid and cystic material associated with peritumoral edema. The patient underwent ventriculoperitoneal shunt and subtotal resection of the lesion. The histological and immunohistochemical studies showed typical features of PMA. The patient started adjuvant therapy with chemotherapy and radiosurgery. She has been asymptomatic for 9 months and has shown no signs of progression of the disease on the follow-up scans.


O astrocitoma pilomixoide (APM) é um raro glioma, recentemente descrito como uma entidade separada, que geralmente se localiza na região hipotalâmica. Anteriormente, o APM era descrito como astrocitoma pilocítico (AP) devido a características semelhantes que ambos apresentam. Estudos recentes permitiram um melhor entendimento do APM, configurando-o como uma entidade separada, embora o APM ainda seja considerado por muitos autores uma variante do AP. O APM é considerado mais agressivo que o AP; no entanto, mais estudos são necessários para um melhor entendimento do comportamento do tumor e, consequentemente, para que neurocirurgiões e neurologistas cheguem a um consenso sobre sua terapêutica. Este estudo apresenta uma paciente de 16 anos que procurou atendimento médico com queixas de dores de cabeça por mais de 6 meses e vômitos nas 2 semanas antecedentes à visita ao médico. Outros sintomas não eram apresentados. O exame físico revelou apenas papiledema bilateral. As imagens de ressonância magnética mostraram uma lesão intraventricular e hipotalâmica de componentes sólido e cístico associados a edema peritumoral. A paciente foi submetida a derivação ventriculoperitoneal e a ressecção subtotal da lesão. Os estudos histológico e imunohistoquímico demonstraram características típicas de APM. A paciente iniciou terapia adjuvante com quimioterapia e radiocirurgia. A paciente está em acompanhamento por 9 meses e, até o momento, manteve-se assintomática e não houve sinais de progressão da doença nos exames de imagem.


Subject(s)
Humans , Female , Adolescent , Astrocytoma , Hypothalamic Neoplasms , Papilledema , Hypothalamus/injuries
19.
Article in English | WPRIM | ID: wpr-741152

ABSTRACT

BACKGROUND: Mixed gliomas, such as oligoastrocytomas (OA), anaplastic oligoastrocytomas, and glioblastomas (GBMs) with an oligodendroglial component (GBMO) are defined as tumors composed of a mixture of two distinct neoplastic cell types, astrocytic and oligodendroglial. Recently, mutations ATRX and TP53, and codeletion of 1p/19q are shown to be genetic hallmarks of astrocytic and oligodendroglial tumors, respectively. Subsequent molecular analyses of mixed gliomas preferred the reclassification to either oligodendroglioma or astrocytoma. This study was designed to apply genetically integrated diagnostic criteria to mixed gliomas and determine usefulness and prognostic value of new classification in Korean patients. METHODS: Fifty-eight cases of mixed OAs and GBMOs were retrieved from the pathology archives of Seoul National University Hospital from 2004 to 2015. Reclassification was performed according to genetic and immunohistochemical properties. Clinicopathological characteristics of each subgroup were evaluated. Overall survival was assessed and compared between subgroups. RESULTS: We could reclassify all mixed OAs and GBMOs into either astrocytic or oligodendroglial tumors. Notably, 29 GBMOs could be reclassified into 11 cases of GBM, IDH-mutant, 16 cases of GBM, IDH-wildtype, and two cases of anaplastic oligodendroglioma, IDH mutant. Overall survival was significantly different among these new groups (p<.001). Overall survival and progression-free survival were statistically better in gliomas with IDH mutation, ATRX mutation, no microscopic necrosis, and young patient age (cut off, 45 years old). CONCLUSIONS: Our results strongly suggest that a genetically integrated diagnosis of glioma better reflects prognosis than former morphology-based methods.


Subject(s)
Astrocytoma , Classification , Diagnosis , Disease-Free Survival , Genetics , Glioblastoma , Glioma , Humans , Necrosis , Oligodendroglioma , Pathology , Prognosis , Seoul
20.
Article in Korean | WPRIM | ID: wpr-728806

ABSTRACT

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation of one of two genes, TSC1 (encoding hamartin, 9q34) and TSC2 (encoding tuberin, 16p13). It invades the central nervous system and various parts of the body, causing various symptoms. Crohn's disease (CD) is a chronic immune-mediated disease that has not been clearly elucidated. It is thought to be caused by an excessive immune response of the body to bacteria that normally exist in the digestive tract with genetic factors. No cases have been reported in which both of the above-mentioned diseases occurred simultaneously. We report a case of CD in a patient with TSC. A 12-year-old boy was brought to our hospital because of abdominal pain. Skin lesions were observed in the TSC. Fundus examination revealed a hamartoma in the right retina. Brain magnetic resonance imaging revealed a subendothelial giant cell astrocytoma (SEGA). On the basis of these findings, he was diagnosed as having TSC. Blood test results showed increased levels of inflammatory markers. On abdominal ultrasonography, his colon walls were observed to be thickened with increased vascularity of the proximal ascending colon, ileocecal valve, and terminal ileum. Colonoscopy revealed discontinuous ulcerations and inflammations of the ileum, IC valve, and cecum, similar to those found in CD. Everolimus was administered orally for the SEGA but was discontinued frequently owing to the exacerbation of CD. The possibility of CD should be kept in mind in patients with TSC considering to undergo treatment for SEGA.


Subject(s)
Abdominal Pain , Astrocytoma , Bacteria , Brain , Cecum , Central Nervous System , Child , Colon , Colon, Ascending , Colonoscopy , Crohn Disease , Everolimus , Gastrointestinal Tract , Giant Cells , Hamartoma , Hematologic Tests , Humans , Ileocecal Valve , Ileum , Inflammation , Magnetic Resonance Imaging , Male , Retina , Skin , Tuberous Sclerosis , Ulcer , Ultrasonography
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