Lateral-type posterior fossa ependymomas in pediatric patients: a national collaborative study.

PURPOSE: To review a multicentric series of lateral-type posterior fossa ependymomas operated in the last ten years and to analyze the factors related to clinical evolution and tumor survival. METHODS: Descriptive, retrospective study. Active members of the Spanish Society of Pediatric Neurosurgery were invited to participate in this multicentric study. Clinical and radiological data were incorporated to an open database. The role of histologic grade, grade of resection, postoperative morbidities, and clinical follow-up was evaluated through bivariate associations (chi-square), Kaplan-Meier's curves (log-rank test), and multivariate analysis (binary logistic regression). RESULTS: Fourteen centers entered the study, and 25 cases with a minimum follow-up of 6 months were included. There were 13 boys and 12 girls with a mean age close to 3 years. Mean tumor volume at diagnosis was over 60 cc. A complete resection was achieved in 8 patients and a near-total resection in 5 cases. Fifteen tumors were diagnosed as ependymoma grade 2 and ten as ependymoma grade 3. Major morbidity occurred postoperatively in 14 patients but was resolved in twelve within 6 months. There were six cases of death and 11 cases of tumor progression along the observation period. Mean follow-up was 44.8 months. Major morbidity was significantly associated with histologic grade but not with the degree of resection. Overall and progression-free survival were significantly associated with complete surgical resection. At the last follow-up, 16 patients carried a normal life, and three displayed a mild restriction according to Lansky's scale. CONCLUSIONS: Lateral-type posterior fossa ependymomas constitute a specific pathologic and clinical tumor subtype with bad prognosis. Gross total resection is the goal of surgical treatment, for it significantly improves prognosis with no additional morbidity. Neurological deficits associated to lower cranial nerve dysfunction are common, but most are transient. Deeper genetic characterization of these tumors may identify risk factors that guide new treatments and stratification of adjuvant therapies.

Multicentric and collaborative study of Spanish neurosurgical management of pediatric craniopharyngiomas: S-PedCPGco / Estudio multicéntrico colaborativo del manejo neuroquirúrgico del craneofaringioma pediátrico en España: S-PedCPGco

Purpose To present a descriptive analysis of pediatric craniopharyngiomas (PedCPG) treated in various Spanish hospitals, defining factors related to recurrence and performing a critical analysis of the results. Methods We undertook a multicenter retrospective review of PedCPG treated between 2000 and 2017. Data collected included epidemiological variables, clinical and radiological characteristics, goal of first surgery, rate of recurrence and its approach, adjuvant treatment, complications and permanent morbidity. Associations were studied between progression and number of progressions and independent variables. Results The study involved 69 children from 8 Spanish hospitals. Most of the tumors invaded several intracranial compartments at diagnosis, with the hypothalamus involved in 41.3% of cases. The first treatment strategy was usually gross total resection (GTR) (71%), with some patients treated with radiotherapy or intracystic chemotherapy. The progression rate after first surgery was 53% in a mean follow-up of 88.2 months (range 7–357). In the GTR group 38.8% of tumors recurred, 40% in the group of subtotal resection or biopsy and 93.3% in the cyst fenestration±Ommaya reservoir group. Mortality was 7.2%. Follow-up period, size of the tumor and goal of first surgery were significantly related with progression. Conclusions Our results in terms of disease control, hormonal or visual impairment and mortality were acceptable, but there are several areas for improvement. Our short-term goals should be to create a national register of PedCPG, reach a consensus about a treatment algorithm, and improve diagnosis of hypothalamic dysfunction to avoid preventable morbidity (AU)

Objetivo Presentar un análisis descriptivo de los craneofaringiomas pediátricos tratados en varios hospitales españoles, definiendo los factores relacionados con la recurrencia y realizando un análisis crítico de los resultados. Métodos Estudio retrospectivo multicéntrico de los craneofaringiomas pediátricos tratados entre 2000-2017. Se recogieron variables epidemiológicas, clínicas y radiológicas, el objetivo de la primera cirugía, la tasa de recurrencia y su abordaje, los tratamientos adyuvantes, así como las complicaciones y la morbilidad permanente. Se estudió la relación estadística entre la progresión y el número de progresiones con las variables independientes. Resultados Se incluyeron 69 niños tratados en 8 hospitales españoles. La mayoría de los tumores se extendían por varios compartimentos intracraneales al diagnóstico, con invasión hipotalámica en el 41,3%. Habitualmente, la primera estrategia de tratamiento fue la resección radical (71%), con algunos pacientes tratados con radioterapia o quimioterapia intraquística. La tasa de progresión tras la primera cirugía fue del 53% en un seguimiento medio de 88,2 meses (rango 7-357). En el grupo de resección radical recurrieron un 38,8% de los tumores, un 40% en el de resección subtotal o biopsia y un 93,3% en el de fenestración quística±reservorio Ommaya. La mortalidad fue de un 7,2%. Las variables relacionadas de forma significativa con progresión fueron el tiempo de seguimiento, el tamaño del tumor y el objetivo de la primera cirugía. Conclusiones Los resultados obtenidos fueron aceptables en control de la enfermedad, secuelas hormonales o visuales y mortalidad, aunque hay varias áreas susceptibles de mejora. Nuestros objetivos a corto plazo deberían estar orientados a crear un registro nacional de craneofaringiomas pediátricos, alcanzar un consenso respecto al algoritmo de tratamiento y mejorar el diagnóstico de la disfunción hipotalámica para evitar morbilidad (AU)

Multicentric and collaborative study of Spanish neurosurgical management of pediatric craniopharyngiomas: S-PedCPG.co.

PURPOSE: To present a descriptive analysis of pediatric craniopharyngiomas (PedCPG) treated in various Spanish hospitals, defining factors related to recurrence and performing a critical analysis of the results. METHODS: We undertook a multicenter retrospective review of PedCPG treated between 2000 and 2017. Data collected included epidemiological variables, clinical and radiological characteristics, goal of first surgery, rate of recurrence and its approach, adjuvant treatment, complications and permanent morbidity. Associations were studied between progression and number of progressions and independent variables. RESULTS: The study involved 69 children from 8 Spanish hospitals. Most of the tumors invaded several intracranial compartments at diagnosis, with the hypothalamus involved in 41.3% of cases. The first treatment strategy was usually gross total resection (GTR) (71%), with some patients treated with radiotherapy or intracystic chemotherapy. The progression rate after first surgery was 53% in a mean follow-up of 88.2 months (range 7-357). In the GTR group 38.8% of tumors recurred, 40% in the group of subtotal resection or biopsy and 93.3% in the cyst fenestration±Ommaya reservoir group. Mortality was 7.2%. Follow-up period, size of the tumor and goal of first surgery were significantly related with progression. CONCLUSIONS: Our results in terms of disease control, hormonal or visual impairment and mortality were acceptable, but there are several areas for improvement. Our short-term goals should be to create a national register of PedCPG, reach a consensus about a treatment algorithm, and improve diagnosis of hypothalamic dysfunction to avoid preventable morbidity.

Initial experience involving treatment and retreatment with carmustine wafers in combination with oral temozolomide: long-term survival in a child with relapsed glioblastoma multiforme.

BACKGROUND: Glioblastomas occur infrequently in children, and the prognosis is better than for glioblastomas seen in adults. Aggressive treatment is justified in pediatric patients. OBSERVATIONS: We present the case of a 6-year-old child with malignant posterior temporal glioma treated with surgery, radiotherapy, local chemotherapy with carmustine wafers, and oral therapy with temozolomide, both at initial diagnosis and at relapse 18 months later. After 6 years, the patient seems healthy with no focal neurologic signs, and imaging studies show no evidence of disease. CONCLUSION: Multimodal therapy was found to have a very positive outcome for a child with malignant glioma.

[Neonatal intracranial haemorrhage secondary to spontaneous rupture of an aneurysm. Illustrative case and literature review]. / Hemorragia intracraneal en el neonato secundaria a rotura aneurismatica espontanea. Caso ilustrativo y revision de la bibliografia.

INTRODUCTION: Intracranial arterial aneurysms are little frequent among the pediatric age group but the frequency is still lower in children under 1 year of age and extremely rare in neonates. We present a case, review literature and propose common characteristics and management of this pathology. CASE REPORT: A term newborn suffered reduction of the level of conscience. After brain ultrasound examination and CT scan was diagnosed with intraparenchymatous hematoma on the 7th day of life. Intraoperative, after evacuation of this hematoma, we discovered, clipped and extirpated a middle cerebral artery aneurysm without any problems. The patient's evolution was satisfactory only presenting a slight paresia of his right arm. CONCLUSION: We review the literature and find 16 cases of neonatal intracranial arterial aneurysms. Mortality rate is about 50%, but keeping in mind better survivals with surgical treatment in the last years. Thus we conclude, when it is possible, in favour of a surgical and precocious treatment of these neonatal aneurysm.

Hemorragia intracraneal en el neonato secundaria a rotura aneurismática espontánea. Caso ilustrativo y revisión de la bibliografía / Neonatal intracranial haemorrhage secondary to spontaneous rupture of an aneurysm. Illustrative case and literature review

Introducción. Los aneurismas intracraneales arteriales son raros en la edad pediátrica, pero mucho más en menores de 1 año, y más extraños aún en neonatos. Presentamos un caso, revisamos la bibliografía y proponemos características comunes, así como recomendaciones terapéuticas. Caso clínico. Recién nacido a término que sufre deterioro del nivel de conciencia y que tras ecografía y tomografía computarizada cerebrales es diagnosticado de hematoma intraparenquimatoso espontáneo al séptimo día de vida. Tras la evacuación del hematoma, intraoperatoriamente se detecta aneurisma de la arteria cerebral media, que se clipa y extirpa sin problemas. El paciente evoluciona de forma favorable, permaneciendo como única secuela a los tres años de seguimiento una ligera paresia en el brazo. Conclusión. Tras revisar la bibliografía, encontramos 16 casos claros de aneurismas neonatales. La mortalidad global es cercana al 50%, pero hay que tener en cuenta que se observan supervivencias mucho mayores tras tratamiento quirúrgico en los últimos años. Por eso concluimos, cuando es posible, a favor de un tratamiento quirúrgico precoz de estas lesiones (AU)

Introduction. Intracranial arterial aneurysms are little frequent among the pediatric age group but the frequency is still ower in children under 1 year of age and extremely rare in neonates. We present a case, review literature and propose common characteristics and management of this pathology. Case report. A term newborn suffered reduction of the level of conscience. After brain ultrasound examination and CT scan was diagnosed with intraparenchymatous hematoma on the 7th day of life. Intraoperative, after evacuation of this hematoma, we discovered, clipped and extirpated a middle cerebral artery aneurysm without any problems. The patient’s evolution was satisfactory only presenting a slight paresia of his right arm. Conclusion. We review the literature and find 16 cases of neonatal intracranial arterial aneurysms. Mortality rate is about 50%, but keeping in mind better survivals with surgical treatment in the last years. Thus we conclude, when it is possible, in favour of a surgical and precocious treatment of these neonatal aneurysm (AU)