Primary central nervous system T-cell lymphoma in children and adolescents: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To study the clinicopathological characteristics, and further understand primary central nervous system T-cell lymphoma (PCNSTCL) in children and adolescents. Methods: Five cases of PCNSTCL in children and adolescents were collected from December 2016 to December 2021 at the First Affiliated Hospital of Zhengzhou University. The clinicopathological characteristics, immunophenotypic, and molecular pathologic features were analyzed, and relevant literatures reviewed. Results: There were two male and three female patients with a median age of 14 years (range 11 to 18 years). There were two peripheral T-cell lymphomas, not otherwise specified, two anaplastic large cell lymphoma, ALK-positive and one NK/T cell lymphoma. Pathologically, the tumor cells showed a variable histomorphologic spectrum, including small, medium and large cells with diffuse growth pattern and perivascular accentuation. Immunohistochemistry and in situ hybridization showed CD3 expression in four cases, and CD3 was lost in one case. CD5 expression was lost in four cases and retained in one case. ALK and CD30 were expressed in two cases. One tumor expressed CD56 and Epstein-Barr virus-encoded RNA. All cases showed a cytotoxic phenotype with expression of TIA1 and granzyme B. Three cases had a high Ki-67 index (>50%). T-cell receptor (TCR) gene rearrangement was clonal in two cases. Conclusions: PCNSTCL is rare, especially in children and adolescents. The morphology of PCNSTCL is diverse. Immunohistochemistry and TCR gene rearrangement play important roles in the diagnosis.

A single-center, retrospective analysis of relapse and progression patterns of primary central nervous system lymphoma: can whole brain radiotherapy be replaced? / 南方医科大学学报

OBJECTIVE@#To analyze recurrence and progression patterns of primary central nervous system lymphoma (PCNSL) in patients without whole brain radiotherapy (WBRT) and assess the value of WBRT in PCNSL treatment.@*METHODS@#This retrospective single-center study included 27 patients with PCNSL, who experienced recurrence/progression after achieving complete remission (CR), partial remission, or stable disease following initial treatments with chemotherapy but without WBRT. The patients were followed up regularly after the treatment for treatment efficacy assessment. By comparing the anatomical location of the lesions on magnetic resonance images (MRI) at the initial diagnosis and at recurrence/progression, we analyzed the patterns of relapse/progression in patients with different treatment responses and different initial status of the lesions.@*RESULTS@#MRI data showed that in 16 (59.26%) of the 27 patients, recurrence/progression occurred in out-field area (outside the simulated clinical target volume [CTV]) but within the simulated WBRT target area in 16 (59.26%) patients, and within the CTV (in-field) in 11 (40.74%) patients. None of the patients had extracranial recurrence of the tumor. Of the 11 patients who achieved CR after the initial treatments, 9 (81.82%) had PCNSL recurrences in the out-field area but within WBRT target area; of the 13 patients with a single lesion at the initial treatment, 11 (84.62%) experienced PCNSL recurrence in the out-field area but within WBRT target area.@*CONCLUSIONS@#Systemic therapy combined with WBRT still remains the standard treatment for PCNSL patients, especially those who achieve CR after treatment or have a single initial lesion. Future prospective studies with larger sample sizes are needed to further explore the role of low-dose WBRT in PCNSL treatment.

Caracterização epidemiológica das neoplasias do sistema nervoso central em pacientes da região Norte do Brasil / Epidemiological characterization of central nervous system tumors in patients from Northern Brazil

Introdução: A incidência de neoplasias que acometem o Sistema Nervoso Central (SNC) tem aumentado gradativamente no mundo. No Brasil, as neoplasias encefálicas primárias são classificadas como a sétima causa de morte entre as neoplasias malignas. O objetivo do presente estudo foi caracterizar o perfil epidemiológico dos pacientes acometidos por neoplasias do SNC em um estado do Norte do Brasil.Métodos: Estudo retrospectivo, onde 196 prontuários, entre 2012 e 2016, de dois hospitais na região Norte do Brasil foram analisados.Resultados: O glioblastoma multiforme foi predominante (30,6%) entre as neoplasias primárias, seguido pelo astrocitoma (12,2%). As neoplasias secundárias, identificadas como metástases encefálicas, corresponderam a 29,9% da amostra, principalmente derivadas de neoplasias do pulmão e mama. A gravidade histológica das lesões neoplásicas foi mais frequente nos homens (p= 0,01). Foi observado que as neoplasias ocorreram com maior frequência a partir da 4º década de vida, exceto nos casos de astrocitoma e meduloblastoma, que foram detectadas principalmente em crianças e adultos jovens.Conclusão: A caracterização dos casos de neoplasias do SNC é de importante para a compreensão da situação atual deste problema de saúde pública na região norte do Brasil.

Introduction: The incidence of central nervous system (CNS) cancer has gradually increased worldwide. In Brazil, primary brain tumors are the seventh leading cause of death among malignant tumors. The objective of the present study was to characterize the epidemiological profile of patients with CNS cancer from a state in northern Brazil.Methods: This retrospective study analyzed 196 medical records between 2012 and 2016 from two hospitals in northern Brazil.Results: Glioblastoma multiforme was predominant (30.6%) among primary tumors, followed by astrocytoma (12.2%). Secondary cancer, defined as brain metastases, accounted for 29.9% of the sample and was mostly associated with lung and breast cancer. The histological severity of neoplastic lesions was more frequent in men (p = 0.01). Cancer occurred more frequently after the fourth decade of life, except in cases of astrocytoma and medulloblastoma, which mostly affected children and young adults.Conclusion: The characterization of CNS tumors is important for understanding the current situation of this public health problem in northern Brazil.

Primary diffuse large B-cell lymphoma of the CNS, with a "Lymphomatosis cerebri" pattern

We describe an unusual case of lymphomatosis cerebri in a middle-aged lady presenting with rapid-onset dementia. The lymphomatous infiltrate, instead of forming mass lesions, percolated throughout the brain parenchyma, which is often missed on a stereotactic biopsy and hence warrants caution and awareness about this entity. The nonspecific symptoms at presentation and a variable picture at imaging make this entity diagnostically challenging.

Epidemiología descriptiva y cambios en la frecuencia de tumores astrocíticos en el Instituto Nacional de Neurología y Neurocirugía de México / Descriptive epidemiology and frequency variations of astrocytic tumors in the National Institute of Neurology and Neurosurgery of Mexico

Resumen: Objetivo: Determinar distribución, localización y cambios de la frecuencia de tumores astrocíticos (TA) en un instituto mexicano de neurología. Material y métodos: Se revisaron los registros institucionales de TA de cinco décadas. Se compararon las relaciones TA/egresos quirúrgicos (EQ) y TA/total de tumores del sistema nervioso central (TSNC) de 1995 a 2014. Resultados: Se analizaron 2 287 TA (1 356 en hombres y 931 en mujeres). El glioma más común fue el glioblastoma multiforme (GBM), que estuvo presente en adultos jóvenes con una frecuencia mayor a la reportada en otros estudios. La relación TA/EQ y TA/TNSC fue similar entre 1995 y 2014. Conclusiones: En general, la frecuencia de TA atendidos en el Instituto es similar a la reportada internacionalmente. No obstante, los casos de TA en el subgrupo de adultos jóvenes con GBM son más frecuentes (40%) que las incidencias reportadas en otros estudios (menores al 5%). No se encontró variación significativa en la frecuencia de TA durante las últimas dos décadas.

Abstract: Objective: To determine distribution, localization and frequency variations of astrocytic tumors (AT) in a Mexican Institute of neurology. Materials and methods: Institutional registries of AT from five decades were analyzed. AT/Surgical discharges (SD) and AT/Central Nervous System Tumors (CNST) from 1995 to 2014 were compared. Results: Two thousand two hundred and eighty-seven AT (1 356 men and 931 women) were analyzed. The most common glioma was glioblastoma multiforme (GBM), found in young adults with a higher frequency to that reported in other studies. Relation of AT/SD, as well as, relation of AT/CNST was similar between 1995 and 2014. Conclusions: In general, the frequency of AT attended at the Institute is similar to that found worldwide, being only higher the number of GBM in younger adults. There was not significant variation in the frequency of AT during the time studied.

Systemic dissemination of glioblastoma: literature review

SUMMARY INTRODUCTION: Glioblastoma (GBM) is the most frequent primary malignant tumor from the central nervous system in adults. However, the presence of systemic metastasis is an extremely rare event. The objective of this study was to review the literature, evaluating the possible biological mechanisms related to the occurrence of systemic metastasis in patients diagnosed with GBM. RESULTS: The mechanisms that may be related to GBM systemic dissemination are the blood-brain barrier breach, often seen in GBM cases, by the tumor itself or by surgical procedures, gaining access to blood and lymphatic vessels, associated with the acquisition of mesenchymal features of invasiveness, resistance to the immune mechanisms of defense and hostile environment through quiescence. CONCLUSIONS: Tumor cells must overcome many obstacles until the development of systemic metastasis. The physiologic mechanisms are not completely clear. Although not fully understood, the pathophysiological understanding of the mechanisms that may be associated with the systemic spread is salutary for a global understanding of the disease. In addition, this knowledge may be used as a basis for a therapy to be performed in patients diagnosed with GBM distant metastasis.

RESUMO INTRODUÇÃO: Glioblastoma (GBM) é o tumor maligno mais comum do sistema nervoso central em adultos. Entretanto, metástase a distância de GBM é um evento extremamente raro. O presente estudo teve o objetivo de realizar uma revisão da literatura para avaliar os possíveis mecanismos biológicos relacionados com a ocorrência de metástase a distância de pacientes com diagnóstico de GBM. RESULTADOS: Os mecanismos que podem estar relacionados com a capacidade de disseminação sistêmica do GBM são a quebra de barreira hematoencefálica (BHE) frequentemente vista em GBM, seja pela doença, seja por procedimentos cirúrgicos, dando acesso aos vasos sanguíneos e linfáticos, associada à aquisição de características mesenquimais de invasividade, resistência aos mecanismos de defesa do sistema imunológico e adaptação a hostilidades dos meios distantes por meio de quiescência. CONCLUSÕES: As células tumorais necessitam vencer diversos obstáculos até a formação de uma metástase distante. Apesar de não totalmente esclarecido, o entendimento fisiopatológico dos mecanismos pelos quais podem estar associados à disseminação sistêmica do GBM é salutar para a compreensão global da doença. Além disso, esse conhecimento pode servir de base para a terapia a ser empregada diante do paciente com diagnóstico de GBM com metástase a distância.

Pathological features of 78 metastatic or multicentric neoplasms involving the central nervous system in dogs / Aspectos patológicos e distribuição de 78 neoplasias metastáticas ou multicêntricas envolvendo o sistema nervoso central de caninos

Secondary neoplasms affecting the central nervous system (CNS) may occur through blood flow or direct extension. This condition occurrence has increased both due to a longer life expectancy of dogs, as well as with the employment of chemotherapeutics, which may increase the survival period and, thus, the odds of the occurrence of metastasis. The aim of this study was to analyze the epidemiological features, such as the age, breed and sex of the animals affected, as well as the gross and microscopic findings of the metastasis from neoplasms involving the CNS of 78 dogs, and, based on the data, estimate the occurrence and frequency of the metastatic or multicentric neoplasms. Females (71.79%) were most affected than males, with an average age of 9.5-years-old and a median of 10-years-old. Most of the cases had a multifocal distribution (73.07%), and epithelial neoplasms (50%) were the most common. Telencephalon was the most affected neuroanatomical region (61.1%). Mammary neoplasms were the most frequent (47.44%), followed by hemangiosarcoma (19.23%), lymphoma (10.26%) and melanoma (6.41%). Less common neoplasms included osteosarcoma, histiocytic sarcoma and lung carcinoma (2.56%). Mammary neoplasms were mainly composed of anaplastic and micropapillary carcinomas, of which only multifocal microscopic lesions were observed.(AU)

Neoplasias secundárias envolvendo o sistema nervoso central (SNC) podem ocorrer por via hematógena ou por extensão direta. A ocorrência desta condição vem aumentando devido à expectativa de vida mais longa dos cães, bem como através do uso de quimioterápicos, os quais podem ampliar a sobrevida e, portanto, as chances para a ocorrência de metástases. O objetivo deste estudo foi analisar os aspectos epidemiológicos, como idade, raça e sexo dos animais afetados, bem como caracterizar os achados macroscópicos e microscópicos de neoplasmas com metástases envolvendo o SNC de 78 caninos e, baseado nestes dados, estimar a ocorrência e a frequência de neoplasias metastáticas ou multicêntricas. Fêmeas (71,79%) foram mais afetadas do que machos, com uma idade média de 9,5 anos e mediana de 10 anos. A maior parte dos casos apresentava distribuição multifocal (73,07%), e neoplasias de origem epitelial (50%) foram as mais frequentes. A localização neuroanatômica mais afetada foi o telencéfalo (61,1%). As neoplasias mamárias foram as mais frequentes (47,44%), seguidas por hemangiossarcoma (19,23%), linfoma (10,26%) e melanoma (6,41%). Osteossarcoma, sarcoma histiocítico e carcinoma pulmonar (2,56%) foram neoplasias menos frequentes. Os tipos de neoplasmas mamários mais observados foram o carcinoma anaplásico e o carcinoma micropapilar, nesses dois tipos as lesões eram multifocais e observadas somente na microscopia.(AU)

The role of surgery in primary central nervous system lymphomas / El rol de la cirugía en los linfomas primarios del sistema nervioso central

ABSTRACT Background Primary central nervous system lymphomas (PCNSL) are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

RESUMO Introducción Los linfomas primarios del sistema nervioso central (LPSNC) son infrecuentes. Tradicionalmente el tratamiento de elección es la quimioterapia. Existe un paradigma de no indicar resección, por el riesgo de déficit neurológico sin aumento de la sobrevida. El objetivo del presente estudio es comparar la sobrevida de pacientes con LPSNC sometidos a biopsia versus resección. Métodos Estudio retrospectivo que incluye 50 pacientes con diagnóstico confirmado de LPSNC tratados en nuestra Institución desde enero de 1994 hasta julio de 2015. Resultados Los pacientes del "grupo resección" mostraron una sobrevida media significativamente mayor respecto a los del "grupo biopsia"; 31 meses versus 14,5 meses respectivamente, p = 0,016. Conclusiones En nuestra serie, los pacientes que con resección quirúrgica de su tumor tuvieron una sobrevida media de 16,5 meses superior que los pacientes biopsiados.

Possível associação entre doença desmielinizante e neoplasias oligodendrogliais: relato de caso / Possible association between demyelinating disease and oligodendroglial tumors: a case report

Desde o primeiro relato de doença desmielinizante associada a tumores cerebrais por Scherer em 1938, inúmeros outros relatos de casos foram publicados fazendo associação desta doença com diferentes tumores primários do sistema nervoso central. Nosso trabalho descreve o caso de uma paciente de 23 anos com duas lesões encefálicas biopsiadas, mostrando inicialmente processo inflamatório desmielinizante que no seguimento desenvolve um oligodendroglioma anaplásico. A partir deste caso, realizamos uma revisão da literatura dessa associação específica, primeiramente publicada por Barnard e Jellinek em 1967, e ressaltamos a importância da diferenciação entre a forma desmielinizante tumefativa de uma neoplasia cerebral verdadeira. (AU)

Since the first report of demyelinating disease associated with brain tumors by Scherer in 1938, several other case reports have been published making association of this disease with different primary tumors of the central nervous system. Our paper describes the case of a 23 year old patient with two brain lesions, biopsied, initially showing a demyelinating inflammatory process that in the follow up develops an anaplastic oligodendroglioma. From this case, we conducted a literature review of this specific association, first published by Barnard and Jellinek in 1967, and emphasize the importance of difference in a tumefactive demyelinating lesions between of true brain neoplasm. (AU)

Linfoma primario del sistema nervioso central: el aporte de las técnicas convencionales de diagnóstico por imágenes / Primary CNS lymphoma: The contribution of conventional diagnostic imaging techniques

El linfoma primario del sistema nervioso central (LPSNC) tiene una presentación predecible en las imágenes convencionales, tanto en pacientes inmunocompetentes como inmunodeprimidos. Analizamos las características imagenológicas que nos permiten reconocer ambos casos y realizamos una revisión de las formas clínicas más relevantes del LPSNC, así como su aspecto en las imágenes obtenidas con técnicas convencionales del Diagnóstico por Imágenes, basándonos en un análisis retrospectivo de nuestros archivos institucionales y la literatura actual. La revisión abarca todas las presentaciones relevantes de esta enfermedad poco frecuente para permitir un diagnóstico temprano, crucial para el adecuado tratamiento.

Primary central nervous system lymphoma (PCNSL) has a predictable imaging appearance on conventional imaging in immunocompetent and immunocompromised patients. The imaging features that enable both types of patients are discussed. The extensive imaging review presented here of the most relevant PCNSL clinical presentations and their imaging appearances using conventional imaging techniques is based on a retrospective analysis of our institutional files and on the current literature. The review covers all of the relevant appearances of this uncommon disease to enable early diagnosis, which is crucial for proper patient management.

Historical distribution of central nervous system tumors in the Mexican National Institute of Neurology and Neurosurgery / Distribución histórica de neoplasias del sistema nervioso central en el Instituto Nacional de Neurología y Neurocirugía de México

Abstract Objective: To determine the frequency of central nervous system (CNS) tumors in the first fifty years of the National Institute of Neurology and Neurosurgery of Mexico Manuel Velasco Suárez (Instituto Nacional de Neurología y Neurocirugía de México, INNN) from 1965 to 2014. Materials and methods: A total of 16 116 institutional records of CNS tumors were analyzed. The frequency and distribution of CNS tumors were evaluated by tumor type, patient age and patient gender. The annual relationship between CNS tumors and surgical discharges (SD) over the last 20 years was estimated. Results: The frequencies of most CNS tumors were consistent with those found worldwide, and the most common tumors were neuroepithelial tumors (33%), particularly astrocytic tumors (67%); meningeal tumors (26%); and pituitary tumors (20%). The incidence of pituitary tumors in these data was twice as high as that reported in other regions of the world, and the relationship between CNS tumors and SD was consistent over time (0.22-0.39). Conclusion: This study summarizes the largest sample of CNS tumor cases analyzed in Mexico and provides an important reference of the frequency of this tumor type in the country. This work will serve as a basis for conducting studies evaluating factors associated with the presence of CNS tumors and for identifying adequate public health interventions.

Resumen Objetivo: Determinar la frecuencia de neoplasias del sistema nervioso central (NSNC) en los primeros 50 años del Instituto Nacional de Neurología y Neurocirugía de México (INNN). Material y métodos: Se analizaron 16 116 registros institucionales de las NSNC, atendidas en el INNN de 1965 a 2014; se estimó su frecuencia y distribución por tipo de neoplasia, edad y género, y se determinó la relación anual de NSNC y egresos quirúrgicos (EQ) en un período de 20 años. Resultados: Las frecuencias de la mayoría de NSNC fueron consistentes con las encontradas a nivel mundial. Las más frecuentes fueron las neuroepiteliales (33%), entre las cuales destacaron las astrocíticas (67%); meníngeas (26%), e hipofisiarias (20%). El número de neoplasias hipofisiarias en esta serie fue dos veces mayor al reportado en otras regiones del mundo y la relación NSNC/EQ fue similar a través del tiempo (0.22-0.39). Conclusión: Ésta es la mayor serie de casos de NSNC analizados en México y proporciona un referente importante sobre la frecuencia de este tipo de neoplasias en el país. Este trabajo servirá de base para llevar a cabo estudios de los factores asociados a la presencia de NSNC e identificar intervenciones de salud pública adecuadas.

Central nervous system lymphoma: magnetic resonance imaging features at presentation / Linfoma do sistema nervoso central: características da imagem de ressonância magnética à apresentação

OBJECTIVE: This paper aimed at studying presentations of the central nervous system (CNS) lymphoma using structural images obtained by magnetic resonance imaging (MRI). METHODS: The MRI features at presentation of 15 patients diagnosed with CNS lymphoma in a university hospital, between January 1999 and March 2011, were analyzed by frequency and cross tabulation. RESULTS: All patients had supratentorial lesions; and four had infra- and supratentorial lesions. The signal intensity on T1 and T2 weighted images was predominantly hypo- or isointense. In the T2 weighted images, single lesions were associated with a hypointense signal component. Six patients presented necrosis, all of them showed perilesional abnormal white matter, nine had meningeal involvement, and five had subependymal spread. Subependymal spread and meningeal involvement tended to occur in younger patients. CONCLUSION: Presentations of lymphoma are very pleomorphic, but some of them should point to this diagnostic possibility.

OBJETIVO: Este trabalho teve como objetivo estudar as apresentações do linfoma do sistema nervoso central (SNC) por meio de imagens estruturais, obtidas por ressonância magnética (RM). MÉTODOS: Foram analisadas as características das imagens por RM, à apresentação, de 15 pacientes diagnosticados com linfoma do SNC em um hospital universitário, entre janeiro de 1999 e março de 2011, pela frequência e por tabulação cruzada. RESULTADOS: Todos os pacientes apresentaram lesões supratentoriais; em quatro (27 por cento) havia lesões infra e supratentoriais. A intensidade do sinal em T1 e T2 foi predominantemente hipo ou isointensa. Lesões únicas foram associadas ao componente de sinal hipointenso nas imagens ponderadas em T2. Seis pacientes apresentaram necrose. Foram encontrados: alteração de sinal da substância branca perilesional em todos os pacientes, acometimento meníngeo em nove e disseminação subependimária em cinco. Disseminação subependimária e acometimento meníngeo tenderam a ocorrer nos pacientes mais jovens. CONCLUSÃO: As apresentações do linfoma no SNC são pleomórficas, mas algumas delas podem apontar para essa possibilidade diagnóstica.

value of touch preparation for rapid diagnosis of brain tumors as an intraoperative consultation

The touch preparation technique is an accurate and rapid method, and when used as intraoperative consultation examination technique it preserves a good amount of tissue for paraffin embedded sections. This study aimed at examining the accuracy of the touch preparation technique by comparing its diagnosis with that of final pathological diagnosis made by microscopic examinations. The diagnoses of 139 central nervous system lesions by touch preparation technique and paraffin-embedded sections were compared. Touch preparation technique diagnosed correctly 118 [84%] of the lesions. However, the technique failed to correctly diagnose 12% of the cases. The highest rate of accurate diagnosis [100%] was observed in five types out of 11 types of tumor examined. However, the technique was not able to diagnose hydatid cysts correctly. The findings indicate that touch preparation technique may be useful in diagnosing tumor type during surgical operations. Touch preparation technique is very accurate for intraoperative diagnosis. However, adequate clinical history, neuroimaging details, and the intraoperative impressions of the neurosurgeons, if provided, help the pathologists to improve the diagnostic accuracy of the technique

Incidence and risk factors for central nervous system relapse in children and adolescents with acute lymphoblastic leukemia

BACKGROUND: Despite all the advances in the treatment of childhood acute lymphoblastic leukemia, central nervous system relapse remains an important obstacle to curing these patients. This study analyzed the incidence of central nervous system relapse and the risk factors for its occurrence in children and adolescents with acute lymphoblastic leukemia. METHODS: This study has a retrospective cohort design. The studied population comprised 199 children and adolescents with a diagnosis of acute lymphoblastic leukemia followed up at Hospital das Clinicas, Universidade Federal de Minas Gerais (HC-UFMG) between March 2001 and August 2009 and submitted to the Grupo Brasileiro de Tratamento de Leucemia da Infância - acute lymphoblastic leukemia (GBTLI-LLA-99) treatment protocol. RESULTS: The estimated probabilities of overall survival and event free survival at 5 years were 69.5% ( 3.6%) and 58.8% ( 4.0%), respectively. The cumulative incidence of central nervous system (isolated or combined) relapse was 11.0% at 8 years. The estimated rate of isolated central nervous system relapse at 8 years was 6.8%. In patients with a blood leukocyte count at diagnosis > 50 x 10(9)/L, the estimated rate of isolated or combined central nervous system relapse was higher than in the group with a count < 50 x 10(9)/L (p-value = 0.0008). There was no difference in cumulative central nervous system relapse (isolated or combined) for the other analyzed variables: immunophenotype, traumatic lumbar puncture, interval between diagnosis and first lumbar puncture and place where the procedure was performed. CONCLUSIONS: These results suggest that a leukocyte count > 50 x 10(9)/L at diagnosis seems to be a significant prognostic factor for a higher incidence of central nervous system relapse in childhood acute lymphoblastic leukemia.

Primary central nervous system lymphoma: A study of clinicopathological features and trend in western India.

Introduction: Primary central nervous system lymphoma (PCNSL) is rare and accounts for 1-2% of all primary intracranial tumors (ICT). There are conflicting reports regarding the increased incidence of PCNSL over the last two decades in both immunocompromised and immunocompetent patients. Aim: This study was designed to study the clinicopathological characteristics of PCNSL and to access the trend of PCNSL at our institute. Materials and Methods: All the histopathologically proven cases of PCNSL were reviewed from January 1997 to December 2009 (13 years). Immunophenotyping was performed on available paraffin-embedded tissue blocks. Immune status was evaluated and human immunodeficiency virus (HIV) serology was performed in all cases. Cerebrospinal fluid (CSF) findings were recorded whenever available. Possibility of secondary involvement by a systemic lymphoma was excluded in every case. Statistical analysis was done using χ2 -test. Results: During the study period (13 years), a total of 4715 cases of ICT were diagnosed, out of which 66 cases were PCNSL, which accounted for 1.4%. The age ranged from 10 to 75 years with a median age of 46 years. All the patients were immunocompetent. Frontal lobe was the most common site of involvement. Diffuse large B-cell lymphoma was the histological pattern in all the cases. CSF involvement was seen in only one case. Conclusions: In this study, no significant increase in the incidence of PCNSL was found at our institute. Association of PCNSL cases with HIV or acquired immunodeficiency syndrome was not found in our study.

Neurocitoma o tumor epitelial disembrioplásico / Neurocytoma or dysembryoplasia epithelial tumor

El neurocitoma o tumor epitelial disembrioplásico (TND) es un tumor infrecuente del sistema nervioso central (SNC) clasificado Grado I, según la OMS, y las series que aparecen en la literatura médica informan de pocos casos. Por considerarse una entidad poco frecuente y por haber asistido en un corto período a 2 pacientes adolescentes uno con recidiva de tumor cerebral que fue reintervenido y donde el diagnóstico patológico inicial se correspondía con un astrocitoma de bajo grado y la otra, llevada a sala de operaciones con el diagnóstico de astrocitoma de alto grado, y donde después de estudios de inmunohistoquímica el diagnóstico final fue de tumor neuroepitelial disembrioplásico extraventricular. Se indicó tratamiento con radiaciones ionizantes. Se decidió revisar la literatura y presentar estos 2 casos

The Neurocytoma or dysembryoplasia epithelial tumor (DET) is an infrequent tumor of the central nervous system (CNS) classified as Degree I according to the WHO and the series appearing in the medical literature report not much cases. It is considered a not much frequent entity and due to the care during a short period to 2 adolescent patients, one of them with relapse of cerebral tumor re-operated on and where the former pathologic diagnosis was in correspondence with a high degree astrocytoma and that after the immunohistochemistry studies the final diagnosis was of a extraventricular dysembryoplasia neuroepithelial tumor. The treatment prescribed was with ionizing radiations. Literature was reviewed and two cases were presented

Improved Outcome of Central Nervous System Germ Cell Tumors: Implications for the Role of Risk-adapted Intensive Chemotherapy

To determine the impact of treatment protocols on the outcome of central nervous system germ cell tumors (CNS-GCTs), we reviewed the medical records of 53 patients who received front-line chemotherapy from September 1997 to September 2006. Pure germinoma, normal alpha-fetoprotein level and beta-human chorionic gonadotropin level <50 mIU/mL were regarded as low-risk features and the others as high-risk. Patients from different time periods were divided into 3 groups according to the chemotherapy protocols. Group 1 (n=19) received 4 cycles of chemotherapy comprising cisplatin, etoposide and bleomycin. Group 2 (n=16) and group 3 (n=18) received 4 cycles of chemotherapy with cisplatin, etoposide, cyclophosphamide and vincristine in the former and with carboplatin, etoposide, cyclophosphamide and bleomycin in the latter. In group 2 and group 3, high-risk patients received double doses of cisplatin, carboplatin and cyclophosphamide. Radiotherapy was given after chemotherapy according to the clinical requirements. The event-free survivals of groups 1, 2, and 3 were 67.0%, 93.8%, and 100%, respectively (group 1 vs. 2, P=0.06; group 2 vs. 3, P=0.29; group 1 vs. 3, P=0.02). Our data suggest that risk-adapted intensive chemotherapy may improve the outcome of patients with malignant CNS-GCTs.

Expresión inmunohistoquímica de la proteína p53 en relación con el grado histológico y riesgo de recidivas en meningiomas intracraneanos

Los meningiomas son neoplasias primarias frecuentes del sistema nervioso central, usualmente benignas y susceptibles de curación mediante cirugía. El grado histológico de la Organización Mundial de la Salud y la extensión de la resección quirúrgica inicial son factores pronósticos determinantes en estos tumores. Sin embargo, una tasa de recidiva cercana al 20 por ciento en meningiomas benignos totalmente resecados plantea la necesidad de considerar nuevos factores pronósticos. Un total de 93 casos fueron seleccionados para el estudio inmunohistoquímico de la proteína p53 en relación con el grado histológico y riesgo de recidivas en los meningiomas. El índice de marcaje inmunohistoquímico de la proteína p53 se incrementó con la progresión del grado histológico (promedios de 37,15 por ciento para el grado I, 44,49 por ciento para el II y 57,81 por ciento para el III) y fue significativamente superior en meningiomas recidivantes (46,19 por ciento vs 32,29 por ciento de los no recidivantes). El punto de corte de 40 por ciento resultó estadísticamente útil para separar a los meningiomas benignos (grado I) de los no benignos (grados II-III) y a los tumores recidivantes de los no recidivantes. De esta manera, un índice de marcaje de la proteína p53 igual o mayor de 40 por ciento puede indicar la posibilidad de un grado más alto en casos histológicamente ambiguos o un riesgo mayor para desarrollar recidivas en meningiomas de un grado histológico en particular (especialmente en tumores benignos)

Meningiomas are frequent primary neoplasms of the central nervous system, usually benign and susceptible to healing through surgery. The histological grade of the World Health Organization and the extension of the initial surgical resection, are determining prognostic factors in these tumors. Nevertheless, a recurrence rate close to 20 percent in benign meningiomas completely resected arises the need of considering new prognostic factors. A total of 93 cases were selected for the immunohistochemical study of p53 protein in relation to the histological grade and the risk of recurrences in meningiomas. The immunohistochemical labeling index of p53 protein increased with the progression of the histological grade (means of 37,15 percent for grade I, 44,49 percent for grade II, and 57,81 percent for grade III) and was significantly superior in recurrent meningiomas (46,19 percent vs 32,29 percent of non recurrent). The cut off of 40 percent became statistically useful to separate the benign meningiomas (grade I) from the non benign (grade II and III) and the recurrent tumors from the non recurrent. In this way, a labelling index of p53 protein equal or superior to 40 percent can indicate the possibility of a higher grade in histologically ambiguous cases or a higher risk to develop recurrencies in meningiomas of a particular histological grade (specially in benign tumors)