Current Situation and Research Progress of Minimally Invasive Treatment on Central Nervous System Tumors / 肿瘤防治研究

Central nervous system (CNS) tumors pose a substantial risk to human health. Conventional therapeutic modalities, including surgical excision, radiotherapy, and chemotherapy, exhibit inherent limitations and adverse effects. Nonetheless, the emergence of minimally invasive surgical techniques and advanced imaging technology have spurred research interest in the realm of neurology toward developing minimally invasive treatments for neurosurgical tumors. These approaches encompass tumor laser interstitial thermal therapy, tumor treating fields, photodynamic therapy, and other related interventions. Minimally invasive treatments offer notable advantages, such as reduced tissue trauma, expedited recovery, and pronounced therapeutic efficacy, rendering them extensively employed in clinical settings. This comprehensive review aims to elucidate accomplishments in the field of minimally invasive CNS tumor treatments while delineating prospective avenues for future development.

A prospective study on the relevance of crush or squash cytology of central nervous system lesions, in the present-day surgery

Background:Theintra-operative cytology in the evaluation of central nervous system (CNS) lesions isasimpleandrapidtechnique, and a usefultool. There is not much literature available on the value of crush cytology during the operation. The current study was planned to assess the feasibility and efficacy of intra-operative squash cytology as standalone method in the rapid intra-operative diagnosis of intracranial tumor.Methods:A prospective observational study was carried out in the department of neurology in collaboration with department of pathology, Medanta: The Medicity, Gurugram, for a period of six months from January 2019 toJune 2019, on randomly selected 60 patients, aged: 18 years and above. Two to three tissue bits, from different sites of the mass, were taken to prepare squash cytology smear.Results:Out of 60 patients operated, 54 had neoplastic and 6 had non-neoplastic lesions. Neoplastic benign lesions were observed in 41 patients and neoplastic malignant lesions were diagnosed in 13 patients. There was 17.67% offering a wrong diagnosis on crush cytology. Out of the 13 cases of meningioma, there was a discrepancy in 3 (three) cases.Conclusions:Crushcytologyisausefuladjuncttothediagnosisinneurosurgicalpractice.Thisstudysuggests and confirms the reliability of squash cytology, but further multicentre studies with larger number of patients may help to decide its use in clinical practice.

A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior ­ Dysplastic Gangliocytoma of the Cerebellum: Case Report

Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.

Papillary tumor in pineal region: Immunohistochemistry meeting the diagnostic challenge

Papillary tumor of pineal region (PTPR) is extremely rare and poses diagnostic challenge with other central nervous system tumors having papillary architecture. Immunohistochemistry is crucial for a definitive diagnosis of PTPR.

Efectos endocrinológicos tardíos del tratamiento oncológico en supervivientes de meduloblastoma / Endocrinological late effects of oncologic treatment on survivors of medulloblastoma

INTRODUCCIÓN: La radioterapia, quimioterapia y la cirugía empleada en el tratamiento de los tumores cerebrales tienen efectos en el eje hipotálamo-hipofisario y pueden resultar en disfunción endocrina hasta en el 96% de los casos. PACIENTES Y MÉTODO: Estudio retrospectivo y descriptivo en pacientes diagnos ticados de meduloblastoma sometidos a tratamiento con quimio y radioterapia en los últimos 20 años en un hospital terciario. Se analizan variables edad, sexo, peso, talla, índice de masa corporal (IMC) al final del seguimiento, estadio de maduración sexual, niveles séricos de TSH y T4 libre, ACTH/cortisol e IGF-1, FSH, LH, estradiol, testosterona, perfil lipídico (colesterol total) y prueba de función dinámica de hormona de crecimiento. RESULTADOS: Muestra total de 23 pacientes. El déficit de hormona de crecimiento es la secuela más frecuente (82 %) seguido de disfunción ti roidea (44,8%) y disfunción puberal (24,1%). Solo se diagnosticó un caso de diabetes insípida y 2 casos de déficit de corticotrofina. CONCLUSIONES: El seguimiento a largo plazo de los supervivientes de meduloblastoma tratados con quimio y radioterapia revela una prevalencia muy alta de disfun ción endocrina, particularmente de deficiencia de hormona del crecimiento y de hipotiroidismo. Creemos oportuna la monitorización y el seguimiento a largo plazo de estos pacientes con el fin de garantizar un manejo terapéutico adecuado de aquellas disfunciones tratables.

INTRODUCTION: Radiation therapy, chemotherapy, and surgery used to treat brain tumors have effects on the hy pothalamic-pituitary-adrenal axis and can result in endocrine dysfunction in up to 96% of cases. PATIENTS Y METHOD: Retrospective and descriptive study in patients diagnosed with medulloblasto ma who underwent treatment with chemo and radiotherapy in the last 20 years in a tertiary hospital. The variables analyzed were age, sex, weight, height, body mass index (BMI) at the end of follow-up, sexual maturity stage, serum levels of TSH and free T4, ACTH/cortisol and IGF-1, FSH, LH, estradiol, testosterone, lipid profile (total cholesterol), and growth hormone dynamic function test. RESULTS: Total sample of 23 patients. Growth hormone deficiency is the most frequent sequelae (82%) fo llowed by thyroid dysfunction (44.8%), and disorders of puberty (24.1%). Only one case of diabetes insipidus and two cases of corticotropin deficiency were diagnosed. CONCLUSIONS: Long-term follow- up of medulloblastoma survivors treated with chemo and radiotherapy reveals a very high prevalence of endocrine dysfunction, especially growth hormone deficiency and hypothyroidism. We believe that monitoring and long-term follow-up of these patients is necessary in order to ensure adequate therapeutic management of those treatable dysfunctions.

Histopathological spectrum of brain tumors: A 4-year retrospective study from a single tertiary care facility

Background: Central nervous system (CNS) tumors are infrequent tumors comprising <2% of all malignancies. However, a rising global trend in these tumors has been observed over the years with new potential risk factors being identified for brain tumors. Objective: The aim of the present study is to highlight the histopathological spectrum of brain tumors in a single tertiary care center in our region. Materials and Methods: This retrospective histopathological analysis of brain tumors was carried out in the Postgraduate Department of Pathology, Government Medical College, Srinagar, Jammu and Kashmir, India, from January 2015 to December 2018. During this period, a total of 117 neurosurgical biopsies were retrieved from the archives of the department. The diagnoses in all the cases were made on histopathological examination of routinely processed tissue. The hematoxylin and eosin (H and E) stained sections in all cases were reviewed by the authors, and diagnosis was confirmed applying the WHO classification 2007. The relative frequency of tumors and the distribution as per age, sex, and location of the lesion were analyzed. Results: A wide range of histopathological spectrum of CNS tumors was observed and was classified according to the WHO classification system of 2007. The primary CNS tumors were graded from Grade I to Grade 1V. Overall tumors of meninges (41.02%) were the most common entity followed by the astrocytic tumors (35.04%). Conclusion: The present study helps to provide information regarding the burden of disease in our area. Despite the use of modern imaging technique that helps in provisional diagnosis of disease, histological examination is gold standard in the diagnosis of varied types of brain tumors. Further utility of immunohistochemistry aids in confirmation of the disease.

Accuracy and utility of intraoperative squash smear cytology in neurosurgical practice

Background: Intraoperative squash smear cytology (ISSC) is a rapid and reliable diagnostic tool that provides guidance to the neurosurgeon for precise targeting of the lesion and its surgical resection. It also helps the surgeon to modify the approach at surgery at times based on the preliminary impression of the lesion on cytology. Objectives: The present study was undertaken to assess the utility of ISSC for cytomorphological diagnosis in a resource-limited setting. The accuracy of the method was assessed by correlating cytological diagnosis with histopathological diagnosis. Materials and Methods: A total of 106 cases of central nervous system (CNS) tumors were examined by squash smear technique for cytological diagnosis which were then compared with histopathological diagnosis provided on paraffin-embedded sections. Results: Cytohistological correlation was available in all the 106 cases included in the study. Of these 106 cases, 96 were concordant with the final histopathological diagnosis, while 10 were discordant. Thus, complete correlation with final histopathological diagnosis was observed in 90.56% of cases. Conclusion: Intraoperative squash smear cytology proved to be a simple, rapid, and inexpensive technique for intraoperative consultation of CNS tumors in the absence of frozen section facility.

Identification of microRNA signature in different pediatric brain tumors

Abstract Understanding pediatric brain tumor biology is essential to help on disease stratification, and to find novel markers for early diagnosis. MicroRNA (miRNA) expression has been linked to clinical outcomes and tumor biology. Here, we aimed to detect the expression of different miRNAs in different pediatric brain tumor subtypes to discover biomarkers for early detection and develop novel therapies. Expression of 82 miRNAs was detected in 120 pediatric brain tumors from fixed-formalin paraffin-embedded tissues, low-grade glioma, high-grade glioma, ependymoma, and medulloblastoma, using quantitative real-time PCR. Low-expression of miR-221, miR-9, and miR-181c/d and over-expression of miR-101, miR-222, miR-139, miR-1827, and miR-34c was found in medulloblastoma; low expression of miR-10a and over-expression of miR-10b and miR-29a in ependymoma; low expression of miR-26a and overexpression of miR-19a/b, miR-24, miR-27a, miR- 584, and miR-527 in low-grade glioma. Cox regression showed differential miRNA expression between responders and non-responders. The most specific were miR-10a and miR-29a low expression in LGG non-responders, miR-135a and miR-146b over-expression in ependymoma non-responders, and miR-135b overexpression in medulloblastoma non-responders. MicroRNAs are differentially expressed in subtypes of brain tumors suggesting that they may help diagnosis. A greater understanding of aberrant miRNA in pediatric brain tumors may support development of novel therapies.

ADHD and Cerebellar Vermis Tumor: DTI Analysis of an Incidental Finding

The increase in number of magnetic resonance imaging (MRI) scans for investigation of neurological diseases in childhood and adolescence leads to increase of incidental findings of central nervous system (CNS) tumors in these stages of life. Among MRI techniques, diffusion-weighted imaging (DWI) and diffusion tensor imaging (DTI) have been increasingly used in brain studies. These images are based on random motion of water molecules in the body, which can change depending on constitution and geometry of biological tissues, as well as the existence of pathologies. This paper reports the use of DTI and DWI to evaluation of a CNS tumor incidentally detected in a patient diagnosed previously with Attention Deficit Hyperactivity Disorder (ADHD). He was diagnosed at age 9 and has been treated with medicines and psycho-pedagogical therapies. At age 15 a MRI detected a cerebellar vermis tumor with a volume of 2 cm3 . Due to parental decision, the patient did not undergo any surgical intervention. During the follow-up period we did not observe significant changes in tumor size or diffusion directions in the tumor and surrounding brain tissues. The main brain tracts presented normal diffusion patterns, both in terms of size and geometry. The DTI analysis showed that lesion was quite homogeneous and isotropic, with no significant restriction of diffusion. There also were no significant diffusion pattern changes in other regions of the brain which possibly could be related to ADHD. So, given the characteristics of lesion and the patient's clinical symptoms, it cannot be directly related to ADHD.

O maior número de exames de Ressonância Magnética (RM) para a investigação de doenças neurológicas na infância e adolescência leva a um aumento de achados incidentais de tumores do Sistema Nervoso Central (SNC) nessas fases da vida. Dentre as técnicas de RM, imagens ponderadas por difusão (DWI) e do tensor de difusão (DTI) vêm sendo cada vez mais empregadas em exames exploratórios. Essas imagens são baseadas no movimento randômico das moléculas de água no organismo, que varia em função da constituição e geometria dos tecidos biológicos, bem como da existência de patologias. Este trabalho relata o emprego das DWI e DTI para avaliação de um tumor no SNC detectado de forma incidental em um paciente com diagnóstico prévio de Transtorno do Déficit de Atenção (TDAH). Ele recebeu esse diagnóstico aos 9 anos, sendo tratado com medicações e suporte psicopedagógico. Aos 15 anos a RM apontou um tumor de 2 cm3 no vermis cerebelar. Por decisão dos pais, não houve qualquer intervenção cirúrgica. Durante o período de acompanhamento não foram observadas mudanças no tamanho do tumor ou direção de difusão na lesão e nas estruturas ao seu redor. Os principais tratos cerebrais apresentaram padrões de difusão normais, em termos de tamanho e geometria. A análise das DTI mostrou que a lesão era bastante homogênea e isotrópica, não apresentando restrição à difusão. Não foram encontradas alterações significantes na difusão em outras regiões do cérebro. Assim, dadas as características da lesão e as manifestações clínicas, não relacionamos o tumor diretamente ao TDAH.

Hematoma postoperatorio en neurocirugía: Presentación de un caso y revisión / Postoperative hematoma in neurosurgery: A case report and review

El hematoma postoperatorio es una de las complicaciones más graves en neuro-cirugía, con una mortalidad que varía entre un 18 y 32%, y una morbilidad significativa. Se han identificado factores de riesgo clínicos, perioperatorios y patológicos que aumentan la probabilidad de sufrir esta complicación. Los autores reportan el caso de un paciente que presentó un hematoma postoperatorio luego de la exéresis de una metástasis cerebral y analizan las posibles causas que llevaron a dicha complicación. Asimismo realizan una exhaustiva revisión no sistemática de la literatura referente a la hemorragia postoperatoria, sus factores de riesgo y manejo perioperatorio.

Postoperative haematoma is one of the most severe complications in neurosurgery, with a mortality rate ranging between 18 and 32% and a significant morbidity. Risk factors clinicals, perioperatives and pathologicals have been indentified that increase the likelihood of this complication. The authors report the case of a patient who suffered a postoperative haematoma after the resection of a brain metastasis and analyze the possible causes that led to this complication. They also perform an exhaustive non systematic review of the literature about postoperative haemorrhage, their risk factors and perioperative management.

Empleo de la radioterapia en el tratamiento a los tumores del sistema nervioso central en niños y adolescentes / Use of radiotherapy in treating central nervous system tumors of children and adolescents

INTRODUCCIÓN: los tumores del sistema nervioso central en los niños y adolescentes constituyen en Cuba la tercera localización en orden de frecuencia de todas las neoplasias; el tratamiento consiste en cirugía, seguida de la irradiación en la mayoría de los casos, y en algunos tipos histológicos, se añade la quimioterapia, o los anticuerpos monoclonales. OBJETIVO: valorar el empleo de la radioterapia en el tratamiento a los tumores del sistema nervioso central en niños y adolescentes. MÉTODOS: se trata de una serie de 98 niños y adolescentes entre 3 y 18 años de edad que fueron irradiados en el Instituto Nacional de Oncología y Radiobiología por padecer: 32 casos tumores del tallo cerebral (32,6 %), 19 meduloblastomas (19,4 %), 11 astrocitomas grado I-II (11,2 %), 9 astrocitomas III-IV y glioblastomas multiformes (gliomas de alto grado, 9,2 %), 6 craneofaringiomas (6,1 %), 6 tumores primitivos neuroectodérmicos (6,1 %), 5 oligodendrogliomas (5,1 %), 4 ependimomas (4,1 %), 3 germinomas (3,1 %), 2 pineoblastomas (2,1 %) y 1 caso de gliomatosis (1 %). Los pacientes fueron irradiados con aceleradores lineales, máscaras termoplásticas de inmovilización, planificación en 3D, conformada, o intensidad modulada, controles dosimétricos y de imágenes; en niños pequeños, se utilizó anestesia. RESULTADOS: 47 pacientes se encuentran vivos (47,9 %), con una tasa de supervivencia de 5 años de 50,2 %, y una mediana de 5,34 años. CONCLUSIONES: la radioterapia resulta ser un arma terapéutica eficaz para tumores del sistema nervioso central en niños y adolescentes.

INTRODUCTION: central nervous system tumors of children and adolescents are the most frequent location of neoplasias in Cuba, their treatment involves surgery followed by radiation in most cases, and chemotherapy or monoclonal antibodies in some histological types of tumors. OBJECTIVE: to evaluate the use of radiotherapy in treating central nervous system tumors of children and adolescents. METHODS: a series of 98 children and adolescents aged 3 to 18 years were treated with radiation at the National Institute of Oncology and Radiobiology including 32 brainstem tumors(32.6 %), 19 medulloblastomas (19.4 %), 11 grade I- II astrocytomas (11,2 %), 9 grade III-IV astrocytomas and multiform glioblastomas (high grade gliomas, 9.2 %), 6 craniopharyngiomas (6.1 %), 6 primitive neuroectodermal tumors (6.1 %), 5 oligodendrogliomas (5.1 %), 4 ependymonas (4.1 %), 3 germinomas (3.1 %), 2 pineoblastomas (2.1 %) and one case of gliomatosis (1 %). The patients were irradiated with linear accelerators, thermoplastic masks of immobilization, conformal 3D planning or modulated intensity, dosimetric and imaging controls whereas anesthesia was used in infants. RESULTS: forty seven patients survived (47.9 %) with a survival rate of 5 years (50.2 %) and a median of 5.34 years. CONCLUSIONs: radiation therapy proves to be an effective therapeutic tool for the treatment of central nervous tumors in children and adolescents.

Expression of ATRX in different central nervous system tumors and the usefulness in differential diagnosis / 实用医学杂志

Objective To investigate the expression of α-thalassemia/mental retardation syndrome X-linked (ATRX) in different central nervous system tumors and the meaningness in differential diagnosis. Methods Expression of ATRX protein was determined by immunohistochemistry in astrocytomas , oligodendrogliomas, glioblastomas, ependymomas and meningiomas. Results Positive expression levels of ATRX in astrocytomas were significantly lower than those in oligodendrogliomas,glioblastomas, ependymomas and meningiomas (all P <0.01). Conclusion The loss of ATRX was mainly occurred in astrocytomas, which could be as a astrocytoma marker in diagnosis.

Tumor rabdoide teratoide en paciente pediátrico: reporte de caso / Teratoid rhabdoid tumor in pediatric patient: case report

El tumor Rabdoide/Teratoide atípico (AT/RT) representa un subtipo tumoral primario de sistema nervioso central bastante raro, con un alto grado de malignidad y de pobre pronóstico (grado IV según OMS) encontrándose principalmente en niños menores de 3 años sin antecedentes de importancia y con cuadros clínicos bastante insidiosos que representa un reto en cuanto al diagnóstico y tratamiento teniendo en cuenta la limitada cantidad de casos reportados a nivel mundial, así como poca literatura disponible. Presentamos el caso de una paciente femenina de 23 meses de edad quien ingresa al servicio de urgencias del Hospital Militar Central (Bogotá, Colombia) en coma con midriasis izquierda, deshidratación grado 2, con escanografía cerebral simple de ingreso donde se observa lesión ligeramente isodensa fronto parieto occipital izquierda con sangrado intralesional que ejerce importante efecto de masa desplazando la línea media 22 mm y produce herniación del uncus, paraclínicos que reportan anemia, trombocitopenia y tiempos de coagulación prolongados, por lo cual se decide ingresar a salas de cirugía como urgencia vital realizando resección de lesión macroscópica de aspecto grisáceo, muy vascularizada, similar al tejido cerebral con sangrado fácil y muy difícil de controlar. 72 horas después requiere nueva intervención quirúrgica por inestabilidad hemodinámica durante el postoperatorio encontrando lesión de similares características, se envían muestras a patología reportando diagnóstico patológico definitivo: tumor Teratoide Rabdoide atípico grado IV OMS.

Atypical teratoid rhabdoid tumor (AT/RT) represents a subtype of malignant CNS neoplasms quite rare, with a high degree of malignancy and poor prognosis (WHO grade IV) found mainly in children younger than 3 years old without medical history of diseases and with Clinical characteristics quite insidious that represents a challenge in the diagnosis and treatment taking into account the limited number of cases reported, as well as little literature available. We report a 23 months of age female who is admitted to the emergency room of the Central Military Hospital (Bogotá, Colombia) in coma with mydriasis, dehydration, In the cranial scan showed injury slightly isodensa fronto-parieto-occipital left with intralesional bleeding that exerts important mass effect by moving the Midline of the brain 22 mm and produces herniation of the uncus, paraclinical reported anemia, thrombocytopenia and prolonged coagulation times, by which it decides to enter rooms for surgery making resection of macroscopic lesion of grayish look, highly vascularized, similar to the brain tissue with easy bleeding and very difficult to control. 72 hours after requires new surgical intervention for hemodynamic instability during the postoperative period finding similar lesion characteristics, samples are sent to Pathology reporting final pathological diagnosis: tumor atypical Teratoid Rhabdoid WHO grade IV by OMS.

El trabajo y los Tumores del Sistema Nervioso Central, en población de la ciudad de México / The work and the Central Nervous System tumors in population of Mexico city

El cáncer es un padecimiento que se incrementa en todo el mundo. Existen factores de riesgo bien identificados, no obstante, su aparición es controvertida. Los Tumores del Sistema Nervioso Central (TSNC) son un grupo de neoplasias, no se conoce su causa, algunos autores señalan exposiciones ambientales, causas genéticas y la ocupación; no existen estudios que evalúen el proceso del trabajo en relación a los riesgos y exigencias laborales y la presencia de los TSNC. Se realizó un estudio de casos y controles. Se incluyeron TSNC del Instituto Nacional de Neurología y Neurocirugía de la Ciudad de México. Se obtuvo información de factores de riesgo ambientales, y de riesgos y exigencias laborales. Se calcularon frecuencia simples, razones de momios e intervalos de confianza al 95%, se consideró estadísticamente significativo una p≤0.05, se realizó regresión logística condicional. Se encontró una asociación entre la presencia de los TSNC y las actividades que demandan niveles altos de atención [RM = 4,25; IC 95% (2,44-7,38)] p = 0,001, así como entre el empleo de fertilizantes e insecticidas y el lugar de residencia (p<0,05). Se debe profundizar en el estudio de las exigencias laborales que demandan intensos niveles de atención y concentración y su relación con el estrés y los daños a la salud.

Cancer is increasing worldwide. There are wellestablished risk factors; however, their appearance is controversial. Tumors of the central nervous system (CNST) are a group of neoplasms of unkown cause, and some authors suggest environmental exposures, genetic factors and occupation as possible causal factors, yet, there are no studies evaluating the risks and demands of work and the presence of CNST. A case-control study was performed, using. CNST from the National Institute of Neurology and Neurosurgery of Mexico City. Information on environmental risk factors, and workplace hazards and demands was obtained. Simple frequency, odds ratios and 95% confidence intervals were calculated, with statistical significance set at p ≤ 0.05, using conditional logistic regression. An association between the presence of CNST and activities that require high levels of care was found [OR = 4.25; (95% CI 2.44 to 7.38)] p = 0.001, and between the use of fertilizers and insecticides and place of residence (p <0.05). Further study of the association between those job requirements that require high levels of attention and concentration and both stress and adverse health effects.

Training pathologist's ability of rapid intraoperative diagnosis of central nervous system tumors / 中华医学教育探索杂志

Training pathologist's ability of rapid intraoperative diagnosis of central nervous system tumors is of great importance to decide ranges of clinical operation and to promote pathological diagnosis level.Pathologist made frozen section,fully understood the clinical data and operation,mastered correct method of slice reading,learned comprehensive analysis,and exercised psychological quality thus to make rapid intraoperative diagnosis correctly.

Atualização terapêutica no tratamento dos craniofaringiomas / Therapeutic update on the treatment of craniopharyngiomas

O craniofaringioma é uma neoplasia de natureza benigna, pouco frequente, responsável por 1 por cento a 3 por cento de todos os tumores intracranianos, sendo a mais frequente neoplasia intracraniana não neuroepitelial na criança. Geralmente o tumor é restrito à região selar e ao III ventrículo, mas, em decorrência da infiltração e frequente aderência ao sistema nervoso central, apresenta comportamento clínico muitas vezes desfavorável, sendo classificado pela Organização Mundial de Saúde (OMS) como grau I, caracterizado como tumor de baixo ou incerto potencial de malignização. As sequelas endocrinológicas ganham destaque devido ao importante impacto na qualidade de vida dos pacientes, na maioria das vezes crianças. O hipopituitarismo e a obesidade hipotalâmica são complicações frequentes, sendo o tratamento desse tumor um grande desafio para endocrinologistas e neurocirurgiões. A combinação da cirurgia, radioterapia e aplicação de drogas e radioisótopos intratumorais tem como objetivo maximizar as chances de cura e tentar minimizar as sequelas pós-operatórias, mas, mesmo assim, a recidiva ainda é frequente. A escolha da modalidade de tratamento mais adequado para os craniofaringiomas é uma decisão difícil e que deve sempre ser individualizada para cada paciente. Com o objetivo de explorar as múltiplas opções terapêuticas para o craniofaringioma, foi realizada revisão na literatura com ênfase nas possibilidades terapêuticas e complicações inerentes ao tratamento dessa patologia.

Craniopharyngioma is an uncommon benign neoplasm, accounting for 1 percent-3 percent of all intracranial tumors, and the most common non-neuroepithelial intracranial neoplasm in childhood. Usually, the tumor is confined to the sellar region and the third ventricle, but due to frequent infiltration and adherence to the central nervous system, it often has an unfavorable clinical behavior. Therefore, it is classified by the World Health Organization (WHO) as a tumor of low or uncertain malignant potential. Endocrine after effects, mainly hypothalamic hypopituitarism, obesity and diabetes insipidus are highlighted due to their important impact on the quality of life of patients, mostly children. Optimal treatment of this tumor is a major challenge for neurosurgeons and endocrinologists. The combination of surgery, radiation, and application of radioisotopes and intratumoral drugs, aims at maximizing the chances of cure with minimal complications. Yet, recurrence is still frequent. Choosing the best treatment modality for craniopharyngiomas is a difficult decision, and it should always be specific for each case. In order to explore the multiple therapeutic options for craniopharyngiomas, we reviewed the literature with emphasis on the therapeutic possibilities and complications inherent to the treatment of this disease.

Brain tumors in the first three years of life: a review of twenty cases

OBJECTIVE AND METHOD: To review the clinical and neuropathological findings as well as the type of therapy and outcome in 20 infants under 3 years-old with central nervous system (CNS) tumor. They were treated at the Department of Neurology, "Hospital das Clínicas" University of São Paulo Medical School, from January 1997 to May 2001. RESULTS: Astrocytoma was the most common histological type (n=7), followed by ependymoma (n=3), medulloblastoma (n=2), craniopharyngioma (n=2) and desmoplastic ganglioglioma (n=2). The location of the tumor was predominantly supratentorial. Mean follow-up time was 20.2 months with recurrence in 7 cases. For each type of tumor we have emphasized the treatment currently recommended. CONCLUSION: Although follow-up time is not sufficient for analyzing survival, a trend of improvement in prognosis was noted, compared to another series of cases from our Institution that had been evaluated before 1990.

OBJETIVO E MÉTODO: Avaliar os aspectos clínicos e histopatológicos, o tipo de tratamento e a evolução de 20 crianças menores de três anos de idade, com o diagnóstico de tumor de sistema nervoso central, que foram tratadas em nossa Instituição no período de janeiro de 1997 a maio de 2001. RESULTADOS: O astrocitoma foi o tumor mais comum (n=7), seguido pelo ependimoma (n=3), meduloblastoma (n=2), craniofaringioma (n=2) e ganglioglioma desmoplásico infantil (n=2). A localização do tumor foi predominantemente supratentorial. A média de seguimento foi 20,2 meses e houve recidiva em sete casos. Para cada tipo de tumor enfatizamos o tipo de tratamento recomendado na atualidade. CONCLUSÃO: Embora o tempo de seguimento não seja suficiente, ainda, para analisar a sobrevida, foi notada nítida tendência a melhor prognóstico em comparação com a casuística proviniente de nossa Instituição que analisou casos abordados antes da década de 90.