The efficacy analysis of allogeneic hematopoietic stem cell transplantation in 48 leukemia patients with central nervous system leukemia / 中华血液学杂志

Objective: To investigate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of leukemia patients also suffering from central nervous system leukemia (CNSL) . Methods: A total of 48 leukemia patients with central nervous system leukemia admitted to our hospital from May 2012 to December 2017 were retrospectively analyzed. Results: ① Including 22 cases of acute lymphocytic leukemia (ALL) , 21 cases of acute myeloid leukemia (AML) , and 5 cases of chronic myelogenous leukemia (CML) . Before transplantation, 19 patients achieved complete remission (CR) , and the rest 29 ones without remission. ②The conditioning regimen used TBI as the main protocol, and 6 patients were combined with whole brain and total spinal cord radiotherapy, 2 with Cyber knife treatment, and children with modified IDA combined with BUCY. ③All 48 patients were successfully transplanted, the median time for leukocyte engraftment was 14 (10-23) days, the median time for platelet transplant 16 (6-78) days. ④Bone marrow was evaluated 28 days after transplantation, all 48 patients reached CR, and DNA testing confirmed that they were all full donor chimerism. ⑤The median follow-up was 14 (2-69) months. Of them, 28 cases survived, 10 relapsed and the rest 3 had recurrence of CNSL after transplantation. One year after allo-HSCT, the overall survival (OS) of CR and non-CR groups were (77.3±10.0) % and (57.6±9.3) % (P=0.409) , respectively, the disease-free survival rates (DFS) were (71.2±11.0) % and (53.9±9.5) % (P=0.386) , respectively. The 1-year OS rates of ALL and AML groups after transplantation were (54.2±10.7) %, (80.1±8.9) %, respectively (P=0.200) , and DFS rates were (49.2±10.8) %, (75.0±9.7) % (P=0.190) , respectively. Conclusion: Allo-HSCT was safe and effective for leukemia patients with CNSL.

The influence of antineoplastic treatment on the weight of survivors of childhood cancer / A influência do tratamento antineoplásico sobre o peso de sobreviventes do câncer na infância

Abstract Purpose: Obesity is a late effect in survivors of childhood cancer and correlates with chronic complications. Survivors of leukemia, brain tumors, and hematopoietic stem cell transplantation are more likely to develop obesity resulting from treatment modalities such as radiotherapy and glucocorticoids. This paper analyzes and integrates the current data available to health professionals in order to clarify strategies that can be used to treat and prevent obesity in childhood cancer survivors. Sources: This is a literature review from on scientifically reliable electronic databases. We selected articles published in the last five years and earlier articles of great scientific importance. Data synthesis: The mechanisms involved in the pathophysiology of obesity in cancer survivors are not completely understood, but it is believed that damage to the hypothalamus and endocrine disorders such as insulin resistance, leptin resistance, and hormone deficiency may be involved. The body composition of this group includes a predominance of adipose tissue, especially in those undergoing hematopoietic stem cell transplant and total body irradiation. The use of body mass index in these patients may lead to an underestimation of individuals' risk for metabolic complications. Conclusion: Early identification of groups using accurate anthropometric assessments, interventional treatment, and/or preventative measures and counseling is essential to minimize the adverse effects of treatment. Physical activity and healthy eating to promote adequacy of weight in the whole population should be encouraged.

Resumo Objetivo: A obesidade é um efeito tardio em sobreviventes do câncer na infância e está correlacionada com complicações crônicas. Os sobreviventes da leucemia, tumores cerebrais e transplante de células-tronco hematopoiéticas têm maior probabilidade de desenvolver obesidade como resultado das modalidades de tratamento, como radioterapia e glicocorticoides. Este artigo analisa e integra os dados atuais disponíveis a profissionais da saúde para esclarecer as estratégias que podem ser usadas para tratar e prevenir a obesidade em sobreviventes do câncer na infância. Fontes: Esta é uma análise da literatura de bases de dados eletrônicas cientificamente confiáveis. Selecionamos artigos publicados nos últimos cinco anos e artigos mais antigos de grande importância científica. Resumo dos dados: Os mecanismos envolvidos na fisiopatologia da obesidade em sobreviventes do câncer não são completamente entendidos, porém acredita-se que o dano no hipotálamo e disfunções endócrinas, como resistência à insulina, resistência à leptina e deficiência hormonal, possam estar envolvidos. A composição corporal desse grupo inclui uma predominância de tecido adiposo, principalmente em pacientes submetidos a transplante de células-tronco hematopoiéticas e irradiação de todo o corpo. O uso do índice de massa corporal nesses pacientes poderá levar a uma subestimação do risco de complicações metabólicas nessas pessoas. Conclusão: A identificação precoce de grupos por meio de avaliações antropométricas precisas, o tratamento intervencional e/ou medidas preventivas e aconselhamento são fundamentais para minimizar os efeitos colaterais do tratamento. A atividade física e alimentação saudável devem ser incentivadas para promover a perda de peso na população em geral.

Primary central nervous system lymphoma: what a neurologist/neurosurgeon should know? / Linfoma primário de sistema nervoso central: o que um neurologista/neurocirurgião deve saber?

Primary central nervous system lymphoma is a rare disease, with bad prognosis. Neurologists and neurosurgeons should be familiar with the diagnostic,and biologic features, as well as the initial management of patients. A correct approach to these patients is mandatory for a better outcome.

Linfoma primário do sistema nervoso central é uma doença rara, com prognóstico ruim. Neurologistas e neurocirurgiões devem estar familiarizados com os aspectos do diagnóstico, características biológicas e do manuseio inicial dos pacientes. A abordagem correta desses pacientes é essencial para obter melhores resultados.

Epilepsia asociada a tumores del sistema nervioso central en niños / Epilepsy associated with central nervous system tumors in children

Introducción: La prevalencia de epilepsia en pacientes con tumores del sistema nervioso central (SNC), producto del tumor per se o secundaria al tratamiento, es mayor que en la población general. El objetivo de este estudio es analizar la frecuencia y características de la epilepsia en pacientes pediátricos con tumores del SNC. Método: Estudio descriptivo retrospectivo, realizado a través de la revisión de fichas médicas de los pacientes pediátricos con tumores de SNC entre los años 2001- 2010 en Hospital Carlos Van Buren de Valparaíso. Resultados: Revisados 97 casos pediátricos de tumores del SNC, dieciocho (18,5 por ciento eran portadores de epilepsia, 2/3 sexo masculino, promedio de edad al diagnóstico del tumor fue 7 años y de primera crisis epiléptica 6 años 7 meses. Un 61 por ciento debutó con crisis epilépticas previo al diagnóstico de tumor. Dieciseis de 18 tumores (88 por ciento) fueron supratentoriales, comprometiendo principalmente el lóbulo temporal (9 de 16). Un 83 por ciento fueron neuroepiteliales, los más frecuentes fueron astrocitomas (50 por ciento). Dieciseis casos (88 por ciento) fueron sometidos a intervención quirúrgica. En relación a la epilepsia, 73 por ciento presentó crisis parciales complejas y 38 por ciento (6 casos de 16) evolucionó con epilepsia refractaria durante el seguimiento. Hubo 3 casos que fallecieron. Conclusión: Las crisis epilépticas, sobre todo las crisis focales fueron una manifestación frecuente en este grupo de pacientes pediátricos con tumores del SNC, especialmente en aquellos de localización supratentorial, ya sea como manifestación inicial y clave para el diagnóstico de tumor o durante su evolución. Un 38 por ciento evolucionó como epilepsia refractaria. El número de intervenciones quirúrgicas y la localización tumoral incidieron en la evolución de la epilepsia. Se enfatiza la importancia de una evaluación acuciosa y búsqueda etiológica, en niños que debutan con crisis epilépticas.

Introduction: Patients with brain tumors, show a higher prevalence of epilepsy than the general population, because of the tumor itself or as a consequence of treatment. The aim of this study is to analyze the incidence and characteristics of epilepsy in patients with brain tumors. Method: Retrospective descriptive study, medical records of pediatric patients with brain tumors between the years 2001-2010 from Hospital Carlos Van Buren were reviewed. Results: From 97 patients with brain tumors, 18 (18.5 percent) presented with epilepsy. Two thirds were males. Mean age for brain tumor diagnosis was 7 years, and for first epileptic seizure 6 years 7 months. In 61 percent epileptic seizures started previous to the tumor diagnosis. Sixteen out of 18 patients (88 percent) had supratentorial, mainly temporal tumors (9/16). 83 percent were neuroepithelial, from which astrocytomas were the most frequent (50 percent). Sixteen patients had surgical treatment (88 percent). Epileptic seizures were complex partial in 73 percent. 38 percent evolved to refractory epilepsy in an average of 5 year follow-up. Discussion: Epileptic seizures, mainly complex partial seizures, were a frequent manifestation of patients with brain tumors, specially supratentorial, as the initial event or in follow up. Thirty eight per cent evolved to refractory epilepsy. Number of surgical interventions and localization of the tumor affected the evolution of epileptic seizures. The relevance of searching etiology in children who have a first epileptic seizure is emphazised.

Evidence-based adjuvant therapy for gliomas: current concepts and newer developments.

The incidence of gliomas is increasing worldwide, including India. Of the 18,820 new cases of primary central nervous system (CNS) tumors diagnosed annually in the United States, gliomas account for over 60% with 30-40% of them being glioblastoma multiforme (GBM), 10% being anaplastic astrocytoma (AA), and 10% being low grade gliomas (LGGs). This is in contrast to one study from West Bengal, India, in which only 7.9% of the brain tumors were GBMs, while 46.8% were astrocytomas. Of all adult primary CNS tumors, GBM is the most common and the most malignant with about 7,000 to 8,000 new cases annually in the United States. Given poor outcomes, a number of treatment approaches have been investigated. Common to these approaches is the use of adjuvant radiation therapy, even as surgery alone, with or without chemotherapy, may be the mainstay for some lower grade and low-risk gliomas. Today, treatment typically involves external beam radiation, with concurrent and adjuvant chemotherapy for more aggressive histologies. Although gliomas are relatively uncommon, active research is ongoing. Results of landmark trials along with some of the recently published trials are presented. These trials and management strategies as well as evolving concepts are found by reviewing over 200 articles in the National Library Medical (NLM) database, PubMed, more than 60 of which are refrenced. Specifically, the database is searched using the following keywords, with various combinations: glioma, low-grade, anaplastic, astrocytoma, oligodendroglioma, oligoastrocytoma, glioblastoma multiforme, chemotherapy, radiation, new concepts, phase III, MGMT, CDX-110 (Celldex), temozolomide, 1p/19q deletion, and bevacizumab.

Therapy for glioma: Indian perspective.

Central nervous system (CNS) are rare neoplasms with considerable heterogeneity and variation. The most common primary lesions of CNS are gliomas. A majority of the data about the demography and management of gliomas has emerged from the west. However, there may be considerable variation in the presentation, behavior, and response to treatment between patients in the western world and the Asian population. This article discusses gliomas with special reference to data from oncology centers in India.

Multiple central nervous system tumors and combined treatment modalities - an illustrative case

Multiple space occupying lesions in the craniospinal axis pose special type of challenges in their management. Surgeries for decompression or total excision of the symptomatic tumors at various sites and at different times are needed. Radiosurgery and administration of chemotherapeutic agents are other modalities for the treatment of such lesions caused by benign or malignant conditions and acute or chronic infections. We report a case of a young woman with central neurofibromatosis harbouring bilateral acoustic neurinomas multiple meningiomas and other cranial and spinal neurofibromas. she underwent radiosurgery for the right vestibular schwannoma [VS] and surgery for the left sphenoidal wing meningioma and a large right C5 nerve root neurofibroma. There was no evidence of tumor necrosis or shrinkage 4 years after the radiosurgery. Brain stem compression increased over 4 years period. she is likely to need futher intervention for symptomatic lesions

Survival improvement with combined radio-chemotherapy in the primary central nervous system lymphomas

The benefits of radio-chemotherapy in HIV-negative primary central nervous system (CNS) lymphomas were analyzed in 40 patients, who received radiotherapy to the brain or craniospinal axis with the total dose of 4460-5940 cGy to the primary tumor. Radiotherapy was followed by systemic chemotherapy, mainly with the cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) regimen, in 16 of the patients. Follow-up ranged from four to 95 months with a median of 15 months. The relapse rate was 72.5%, and 83% of the relapses occurred within the radiation field. Median survival was 19 months and the two-year survival rate was 41%. Survival was significantly influenced by treatment method and radiation dose when measured by univariate analysis; median survival and the two-year survival rate was 29 months and 63% after radio-chemotherapy, while 13.5 month and 29% after radiotherapy alone (p= 0.027), and 22 months and 49% with doses of 50 Gy or more, but 12.5 months and 13% with doses less than 50 Gy (p=0.009). However, statistical significance was lost in multivariate analysis. These results might suggest the short-term efficacy of radio-chemotherapy, however, cautious observation is needed to confirm long-term effects.

Ganglioma intraventricular: reporte de un caso y revisión de la literatura / Intraventriculary ganglioma: report of a case and revision of the literatura

Se presenta un caso clínico de un joven masculino de 14 años con un LOE intraventricular, cuyo diagnóstico Histopatológico resultó ser un ganglioma del 3er ventriculo. Fue removido quirúrgicamente en forma total, sin recibir radioterapia ni quimioterapia. Se hace revisión de la literatura acerca de esta infrecuente Neoplasia del Sistema Nervioso Central, siendo a nuestro conocimiento el primer caso descrito en el país

Cuadro clínico patológico y sobrevida en tumores primarios malignos del sistema nervioso central / I square clinical pathological and sobrevida in wicked primary tumors of the central nervous system

Con la finalidad de identificar el cuadro clínico-patológico y la sobrevida actuarial a 5 años de acuerdo al tipo de tratamiento se analizó retrospectivamente información de 19 pacientes con diagnóstico de tumores primarios malignos del sistema nervioso central, atendidos en el Hospital Belén, Trujillo, Perú, desde 1966 a 1995. La serie estuvo conformada por 15 hombres y 4 mujeres (H:M igual 3,7:1), con una edad promedio de 27,9 ñ 22,8 años (rango, 1 a 69 años). Cefalea (74 por ciento), déficit neurológico motor (47 por ciento) y vómitos (47 por ciento) fueron las molestias más frecuentes, y en general predominaron las manifestaciones de hipertensión endocraneana sobre las focales. Todos los tumores estuvieron localizados en el encéfalo, y el compartimiento más afectado el supratentorial (79 por ciento). El glioblastoma multiforme (n igual 7) fue la neoplasia más común, seguido por el astrocitoma anoplásico (n igual 6) y el meduloblastoma (n igual 3). Otros tumores fueron ependimona maligna (n igual 1), astrocitoma fibrilar (n igual 1) y fibrosarcoma (n igual 1). La sobrevida actuarial a 5 años luego del tratamiento con cirugía sola (n igual 8), cirugía más radioterapia (n igual 7) y cirugía más radioterapia y quimioterapia (n igual 4) fue de 0 por ciento, o por ciento y 25 por ciento, respectivamente. La sobrevida actuarial a 5 años ne la serie total fue de 5,6 por ciento. Los esquemas combinados de tratamiento pueden prolongar la supervivencia, sin embargo el pronóstico a largo plazo continúa siendo sombrío para los pacientes con tumores primarios malignos del sistema nervioso central.

Linfoma primario del sistema nervioso central en pacientes no inmunodeprimidos. Estudio clínico radiológico comparativo con gliomas / Primary central nervous system lymphoma. Clinical comparative radiological gliomas

Los linfomas primarios del sistema nervioso central (LPSNC) son un grupo infrecuente de neoplasias que a diferencia del tratamiento eminentemente quirúrgico de los gliomas, en el LPSNC es a base de radioterapia y quimioterapia. En el presente estudio se evaluó la posible presencia de signos clínicos o radiológicos que permitan diferenciar el LPSNC de los gliomas. Se revisaron retrospectivamente la historia clínica y la tomografía computada cerebral de 4 pacientes con LPSNC, los resultados se compararon con otros estudios en pacientes con gliomas. El cuadro clínico de los pacientes con LPSNC fue similar a lo comunicado en los pacientes portadores de glioma. La imagen tomográfica de lesión isoo más frecuentemente hiperdensa, circunscrita, de textura uniforme y rodeado de escaso edema, con captación uniforme de contraste, fue el hallazgo encontrado en nuestros pacientes. Estas características tomográficas se presentan también en los gliomas, pero son poco frecuentes; esto nos permitirá sospechar de un LPSNC y proceder a confirmarla, idealmente con biopsia estereotáxica, por tener implicancias pronósticas y terapéuticas diferentes

The primary central nervous system lymphoma (PCNSL) are an infrequent group of neoplasm which differ in the treatment with gliomas which is basically surgical while for PCNSL it is chemo and radiotherapy. In the present study we evaluated the possible presence of clinical or radiological signs that may allow to differentiate both entieies. The clinical history and brain computed tomography (CT) of 4 cases with PCNSL were retrospectively and compared with other cases with gliomas. The clinical findings were similar in both groups. The CT scan findings in PCNSL included an isodense or hyperdense (more frequent), circumscribed, uniform texture surrounded by scant edema with homogeneus uptake of the contrast material. These findings have also been reported in gliomas but are very infrequent. With the previously described CT scan findings we could presume the diagnosis of PCNSL an comfirm it ideally with stereotactic biopsy since it has different prognostic and therapeutic connotation.

Linfoma primario del sistema nervioso central en pacientes no inmunodeprimidos. Estudio clínico radiológico comparativo con gliomas / Primary central nervous system lymphoma

Los linfomas primarios del sistema nervioso central (LPSNC) son un grupo infrecuente de neoplasias que a diferencia del tratamiento eminentemente quirúrgico de los gliomas, en el LPSNC es a base de radioterapia y quimioterapia. En el presente estudio se evaluó la posible presencia de signos clínicos o radiológicos que permitan diferenciar el LPSNC de los gliomas. Se revisaron retrospectivamente la historia clínica y la tomografía computada cerebral de 4 pacientes con LPSNC, los resultados se compararon con otros estudios en pacientes con gliomas. El cuadro clínico de los pacientes con LPSNC fue similar a lo comunicado en los pacientes portadores de glioma. La imagen tomográfica de lesión iso o más frecuentemente hiperdensa, circunscrita, de textura uniforme y rodeado de escaso edema, con captación uniforme de contraste, fue el hallazgo encontrado en nuestros pacientes. Estas características tomográficas se presentan también en los gliomas, pero son poco frecuentes; esto nos permitirá sospechar de un LPSNC y proceder a confirmarla, idealmente con biopsia estereotáxica, por tener implicancias pronósticas y terapéuticas diferentes

Perspectives in the management of central nervous system tumours.

A general review and update in the management of CNS tumours using medulloblastoma as the main model is given in this article. Special emphasis has been placed on the benefits of combined modality treatment for brain tumours. The pathogenesis and management of these tumours is discussed and recommendations made for treatment in developing countries.

Biópsia estereotáxica para linfomas primários do sistema nervoso central / Stereotaxic biopsy for primary lymphomas of the central nervous system

Os linfomas primários do SNC säo ratos, mas sua incidência tem aumentado nos últimos anos. Estes tumores frequentemente se localizaram nos gânglios de base e tálamo ou se apresentam de forma multifocal. O tratamento atual para eles é a radioterapia, associada ou näo a quimioterapia. Devido à sua extremamente baixa morbidade, a biópsia estereotáxica é o método ideal para determinar o diagnóstico histológico em pacientes com suspeita de linfomas do SNC. Os autoes apresentam estudo de 49 casos de linfomas primários do SNC, diagnosticado por biópsia esterotáxica