Ependimomas intracraneales pediátricos: Si el objetivo es la curación, ¿cuál es el verdadero costo? / Pediatric Intracranial Ependymomas: If the Goal Is Cure, What Is the True Cost?

Introducción: Para caracterizar mejor la morbilidad neurológica después de una cirugía de resección máxima seguida de radioterapia en niños con ependimoma infratentorial, decidimos estudiar el estado neurológico prequirúrgico y compararlo con las evaluaciones postoperatorias a corto y largo plazo. Al mismo tiempo realizamos un estudio de sobrevida libre de progresión (SLP) tumoral para conocer qué factores tienen mayor impacto en el pronóstico de este tipo de lesiones. Métodos: Se realizó un estudio de cohorte retrospectivo donde se incluyeron todos los pacientes pediátricos con diagnóstico de ependimoma infratentorial. Se identificaron los distintos factores de riesgo y se observó cómo evolucionaron en el tiempo. Los pacientes se siguieron por un mínimo de 24 meses para el análisis de supervivencia. Resultados: Se analizaron 26 pacientes pediátricos con ependimomas de fosa posterior entre 2008-2019. Encontramos una diferencia estadísticamente significativa entre el FSS (Escala funcional neurológica) prequirúrgico y el FSS postoperatorio inmediato (p=0.03), sin embargo esta diferencia se pierde cuando comparamos el prequirúrgico con el FSS posterior al año (p=0.07).La exéresis total de la lesión tiene un efecto protector en la SLP tumoral (p=0.02), mientras que haber requerido más de 3 cirugías afecta negativamente la SLP tumoral (p=0.04), al igual que la localización lateralizada del tumor (p=0.04). Conclusión: La exéresis completa de los ependimomas de fosa posterior continúa siendo el factor pronóstico más importante para la sobrevida libre de progresión tumoral. El deterioro neurológico inmediato producido a causa del procedimiento quirúrgico parecería mejorar en la evaluación a largo plazo.

Introduction: To better characterize the neurological morbidity after maximal resection surgery followed by radiotherapy in children with infratentorial ependymoma, we decided to study the preoperative neurological status and compare it with short and long-term postoperative evaluations. At the same time, we conducted a tumor progression-free survival (PFS) study to find out which factors have the greatest impact on the prognosis of this type of injury. Methods: A retrospective cohort study was carried out in which all pediatric patients with a diagnosis of infratentorial ependymoma were included. The different risk factors were identified and it was observed how they evolved over time. Patients were followed for a minimum of 24 months for survival analysis. Results: 26 pediatric patients with posterior fossa ependymomas were analyzed between 2008-2019. We found a statistically significant difference between the presurgical FSS (Functional Status Scale) and the immediate postoperative FSS (p = 0.03), however this difference is lost when we compare the presurgical with the FSS after one year (p = 0.07).Total excision of the lesion has a protective effect on tumor PFS (p = 0.02), while having required more than 3 surgeries negatively affects tumor PFS (p = 0.04), as does the lateralized location of the tumor (p = 0.04). Conclusion: Complete excision of posterior fossa ependymomas continues to be the most important prognostic factor for tumor progression-free survival. The immediate neurological deterioration produced by the surgical procedure would appear to improve on the long-term evaluation

The utility of texture analysis in differential diagnosis of medulloblastomas and ependymomas / 实用放射学杂志

Objective To analyze magnetic resonance images of medulloblastomas and ependymomas by texture analysis method, and evaluate the texture characteristics of two tumors by support vector machine (SVM).Methods 58 tumors were analyzed retro-spectively,including 31 medulloblastomas and 27 ependymomas,which had been confirmed with operation and pathology.Texture analysis was performed onthese tumors.Results The 5 texture parameters extracted from the gray level co-occurrence matrix and at least 3 parameters which had statistical significance between the two different classes of data sets(P<0.05).For all data sets,the variance parameters of gray value of ROI region have statistical significance.The accuracy of SVM test was (86.15±4.16)%,which had remarkable significance between the ependymomas and medulloblastomas.Conclusion The analysis of texture features can pro-vide a lot of quantitative information,and can be a potential method for the differential diagnosis of medulloblastomas and ependy-momas.

Clinicopathological analysis of supratentorial parenchymal ependymomas / 中国基层医药

Objective To study the clinicopathological characteristics and the immunohistochemistry of supratentorial parenchymal ependymoma.Methods The clinical and pathological data of 12 patients with supratento-rial parenchymal ependymoma were analyzed,and the tissue slices were immunohistochemically stained.Results The average age of the patients was 29 years old.Most of the lesions occurred in the frontal and parietal lobes,with increased intracranial pressure as the main clinical symptom,including headache,dizziness,nausea and vomiting.The histology showed that the structure of supratentorial parenchymal ependymoma was consistent with that of infratentorial ependymoma.The tumor cells were oval or spindle shaped,diffuse or patchy distribution,small volume,large nuclear staining deep,and were arranged in Flexner-Winterseiner or Homer-Wright.The immunohistochemistry showed that GFAP,vimentin and S-100 were mostly positive,EMA was partly positive and CD56 and NSE were rarely positive. Conclusion Supratentorial parenchymal ependymoma can occur in any part of the supratentorium,mostly in the fron-tal and parietal lobes.It has a specific pathological morphology and immune phenotype,without any characteristic symptoms in clinical,which is easy to be misdiagnosed.Its diagnosis depends on the pathological examination and the prognosis is largely related to whether the surgical resection is complete or not.

Prognostic factors for 31 cases of intracranial ependymomas after postoperative radiotherapy / 中华放射医学与防护杂志

Objective To evaluate the prognostic factors and treatment results of intracranial ependymomas (EPs).Methods Thirty-one intracranial EPs patients who received postoperative radiotherapy in Beijing Shijitan Hospital between January 2009 and June 2012 were analyzed retrospectively.Twenty-two males and 9 females had an average age of 18 years (range 3-60 years).Seventeen patients received gross total resection (GTR) while fourteen received subtotal resection (STR).Median total radiation dose was 53.9 Gy (48.6-60 Gy).The three-year and five-year progress-free survival (PFS) and overall survival (OS) were estimated with the Kaplan-Meier method.Univariate analysis was performed using eight clinical and dosimetric factors by Log-Rank testing.Cox proportional hazards model was used to identify the independent prognostic factors correlated to EPs.Results The median time of follow-up was 51 months.At the endpoint of the follow-up period,7 patients experienced tumor recurrence:5 had a local recurrence (LR) and 2 had both LR and distant recurrence.Six patients died,4 of which had cases of pediatric infratentorial anaplastic EPs.The three and five-year progress-free survival (PFS) were 80.6％ and 75.9％.Overall survival (OS) at three-year and five-year were 83.9％ and 76.2％,respectively.Univariate analysis showed a more favorable prognoses in terms of three-year PFS,five-year PFS and OS for GTR compared to STR (x2 =4.685,6.311,4.238,P ＜ 0.05).Besides,a more favourable univariate outcome in terms of five-year PFS was evident in patients when the total radiotherapy dose was ＞ 55 Gy compared to ≤55 Gy (x2 =4.210,P ＜ 0.05),and no severe radiotherapy complications occurred.Conclusions Surgery is the major treatment method,while adjuvant radiotherapy is important for subtotal resection and anaplastic EPs patients.Surgical resection and radiotherapy dose were relevant to prognosis.

The utility of apparent diffusion coefficient in differential diagnosis of common pediatric cerebellar tumors / 实用放射学杂志

Objective To investigate the assessment value of diffusion-weighted magnetic resonance imaging (DWI)and apparent diffusion coefficient (ADC)in the differential diagnosis of common cerebellar tumors of children.Methods 45 patients with cerebel-lar tumors were retrospectively analyzed,including 23 medulloblastomas,14 astrocytomas,and 8 ependymomas .The ADCmin of parenchyma of different tumors were measured and Receiver operating characteristic (ROC)curve were delineated.The optimum ADC value for differential diagnosis of common cerebellar tumors of children was analyzed and determined.Results The mean ADC-min of medulloblastoma (0.497 ± 0.023 )× 10 -3 mm2/s was lowest,the mean ADCmin of astrocytoma (1.572 ± 0.145 )× 10 -3 mm2/s was highest,while that of ependymoma was (0.784 ± 0.037 )× 10 -3 mm2/s.The optimum ADC to distinguish ependymomas from medulloblastomas was 0.61 1 × 10 -3 mm2/s (100% sensitivity and 95.7% specificity).The optimum ADC to distinguish low-grade astrocytomas from ependymomas was 1.064×10 -3 mm2/s (92.9% sensitivity and 100% specificity).Conclu-sion ADC is very helpful with differential diagnosis of common cerebellar tumors of children.

Avaliação da evolução pós-operatória dos ependimomas intramedulares / Post-operative outcome evaluation of intramedullary ependymomas

OBJETIVO: Avaliar a eficácia da técnica microcirúrgica utilizada para ressecção completa dos ependimomas intramedulares sem agregar déficits aos pacientes, em especial, aos pacientes funcionalmente independentes no pré-operatório. MÉTODO: Este estudo avaliou, segundo a escala funcional de McCormick, a evolução pós-operatória de uma série de 20 pacientes submetidos à ressecção microcirúrgica total para ependimomas intramedulares. RESULTADOS: A ressecção completa foi atingida em 19 dos 20 pacientes (95%) e somente um paciente apresentou piora clínica (5%). Nos pacientes com independência funcional pré-operatória, com McCormick grau I e II, não houve piora clínica e todos os tumores foram ressecados completamente. No subgrupo de pacientes grau II, a média do status funcional pós-operatória apresentou melhora estatisticamente significativa. Nenhum paciente com grau IV melhorou após o tratamento cirúrgico. CONCLUSÃO: O tratamento cirúrgico foi eficaz para ressecar completamente os tumores sem agregar déficits neurológicos na maioria dos pacientes. Nos pacientes com McCormick graus I e II pré-operatórios a cirurgia deve ser indicada no momento do diagnóstico.

OBJECTIVE: Evaluate the efficacy of the microsurgery technique used aiming total tumor resection without causing neurological deficits, with special attention to the patients with good functional status before surgery. METHOD: This study has evaluated according to the McCormick functional scale the surgical outcome of a series of twenty patients with intramedullary ependymomas submitted to microsurgical resection. RESULTS: Total surgical resection was achieved in 19 of the 20 patients (95%). Only one patient experienced clinical worsening (5%). Patients classified as McCormick grade I and II who were independent pre-operatively remained so in the post-operative period and had their tumors completely removed. In grade II patients there was a significant improvement in their post-operative status. None of the grade IV patients improved after surgical treatment. CONCLUSION: Surgical treatment has proven to be efficient in completely removing tumors without adding neurological deficits in most patients. In patients with McCormick grades I and II pre-operatively surgery should be indicated early in the diagnosis.

Surgical management of intramedullary spinal ependymomas / Abordagem cirúrgica dos ependimomas intramedulares

BACKGROUND: Spinal intramedullary ependymoma is a rare disease with a wide range of clinical presentation, generally requiring surgical treatment. OBJECTIVE: Report our experience and present our surgical technique to achieve total resection and cure. METHOD: We present 12 consecutive cases of intramedullary ependymomas operated between 2000 and 2008 by the senior author (HT). The functional scale proposed by McCormick was used to evaluate the patients' neurological status. RESULTS: Age at presentation varied from 18 to 55 (average 36) years. All tumors had a benign histology. Four (33 percent) patients were male and eight (67 percent) were female. According to the site of presentation, six (50 percent) were localized at the cervical region (including two at the cervicomedullary junction, two at the cervico-thoracic junction and two exclusively at the cervical level), four at the thoracic level and two at the conus/ cauda equina. Dyshestesia was a common finding at the neurological exam in eight patients (67 percent). Total resection was achieved in all cases. Six patients showed neurological improvement postoperatively, whereas the other six remained stable. CONCLUSION: Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors with minimal morbidity and maximum functional recovery.

INTRODUÇÃO: Os ependimomas intramedulares são lesões raramente encontradas na prática neurocirúrgica, tendo apresentação clínica variada, geralmente requerendo tratamento cirúrgico. OBJETIVO: Relatar nossa experiência e discutir a técnica microcirúrgica para a ressecção total e conseqüente cura destas lesões. MÉTODO: Apresentamos uma série de 12 casos de ependimomas intramedulares operados sucessivamente entre 2000 e 2008 pelo autor sênior (HT). A evolução neurológica foi avaliada através da classificação funcional de McCormick. RESULTADOS: A idade dos pacientes variou de 18 a 55 anos (média de 36 anos). Todos eram histologicamente benignos. Quatro (33 por cento) eram do sexo masculino e oito do feminino (67 por cento). A localização das lesões esteve distribuída da seguinte forma: seis casos (50 por cento) na região cervical (sendo dois na transição cérvico-bulbar, dois na região cérvico-torácica e dois na região cervical isoladamente), quatro na região torácica e dois no nível do conus-cauda-eqüina. Disestesias estavam presentes em oito pacientes no pré-operatório (67 por cento dos casos). A ressecção total foi atingida em todos os casos. Em seis casos (50 por cento), houve melhora dos sintomas neurológicos, enquanto que nos outros seis houve manutenção do quadro clínico. CONCLUSÃO: O conhecimento anatômico e de técnicas microcirúrgicas adequadas permite a ressecção total destas lesões propiciando a cura da doença com mínima morbidade e máxima recuperação funcional.

Factores pronósticos y sobrevida de pacientes pediátricos con ependimomas / Prognostic and survival factors among pediatric patients with ependymomas

Antecedentes: El ependimoma es el tercer tumor intracraneano más frecuente en pediatría. Los factores que influyen en el pronóstico son la localización, la edad del paciente, la resección quirúrgica y el tratamiento con radioterapia. Recientemente se ha descrito la presencia de alteraciones cromosómicas en el tumor como factor de mal pronóstico. El objetivo de esta investigación fue determinar la sobrevida de pacientes pediátricos con ependimoma de acuerdo con los diferentes factores pronósticos. Métodos: Se revisaron los expedientes de pacientes pediátricos con ependimoma de 1996 a 2005. Se determinaron las alteraciones cromosómicas mediante hibridación genómica comparativa. Se calculó la sobrevida de acuerdo con el método Kaplan-Meier y prueba de log rank de acuerdo a cada factor pronóstico. Se calculó el riesgo de morir para cada factor de acuerdo con la razón de momios. Resultados: Se incluyeron 24 pacientes. La sobrevida global fue de 58.04%. La presencia de alteraciones cromosómicas, particularmente en el cromosoma 21, afectó significativamente la sobrevida. La edad menor a cinco años, la histología anaplásica, la quimioterapia diferente a ifosfamida-carboplatino-etopósido y la resección parcial aumentaron el riesgo de morir. Conclusiones: Se confirman los factores descritos anteriormente en la literatura, incluyendo alteraciones cromosómicas. Se describe un nuevo desbalance en el cromosoma 21 en 30% de los pacientes.

BACKGROUND: Ependymomas constitute the third most common intracranial tumors in children. Risk factors include age, location, extent of surgical excision, and radiation therapy. Recently, chromosomal imbalances have been described. OBJECTIVE: Determine global survival of patients with ependymomas according to different prognostic factors. METHODS: We reviewed the medical charts of every pediatric patient with ependymoma from 1996 to 2005. Genomic imbalances were determined using comparative genomic hybridization (CGH). Survival was calculated using the Kaplan and Meier method. We used the Log Rank test for each risk factor. Death risk was calculated by odds ratio (OR). RESULTS: We included 24 patients. Global survival was 58.04%. The presence of chromosomal imbalances, particularly in chromosome 21, significantly affected survival Being under 5 years of age, anaplastic histology, chemotherapy other than ICE (ifosfamida-carboplatin-etoposide) and partial resection increased the risk of death. CONCLUSIONS: Known risk factors were confirmed in our study, including chromosomal imbalances. We describe a new chromosomal imbalance in chromosome 21 among 30% of study participants.

A Case of Giant Ependymomas of the Cauda Equina associated with Thoracic Intradural Spinal A-V Malformations

Spinal ependymomas originate in the ependymal lining and grow rather slowly which allows them to reach considerable size and length. The great majority of primary tumors of the cauda equina are ependymomas and show a rather marked predilection for the male. Histologically ependymomas are benign but merastasis and anaplasia have been reported. The basic architectural structures of the ependymoma are the ependymal rosettes and pseudo-rosettes. Intradural spinal arteriovenous malformmations are relatively rare lesions and found only at autopsy or incidentally during surgical operations before the advent of investigative procedure. Development of myelography and spinal angiography have shown that vascular malformations of the spinal cord are not rare. Male is more predominant than female. The etiology and pathophysiological mechanisms are uncertain but many authors have believed that congenital, trauma, inflammations, tumoral processes, vasculitis and abnormal posture are influenced on A-V malformations of the spinal cord. No reports have been noticed about giant ependymomas of the cauda equina combined with spinal A-V malformations. We have experienced a case of giant ependymomas of the cauda equina associated with thoracic intradural spinal A-V malformations in forty-two year-old male and reviewed the literatures.