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.

Regresión espontánea de quiste coloide de tercer ventrículo en paciente pediátrica / Spontaneous resolution of colloid cyst of the third ventricle in a pediatric patient

Los quistes coloides son lesiones intracraneales benignas localizadas mayoritariamente en el III ventrículo en la región de los agujeros de Monro. La mayoría se presentan entre la tercera y cuartas décadas de la vida, y son poco frecuentes por debajo de los 10 años. Presentamos el caso de una niña de 2,5 años que presentaba un quiste coloide incidental que fue seguido de manera conservadora, observándose en los estudios de resonancia magnética una progresiva regresión en su tamaño. Realizamos una revisión de los 4 casos similares publicados con anterioridad (AU)

Colloid cyst are benign intracranial lesions located in the III ventricle in the region of the foramen of Monro. The majority present in the third and fourth decades of life and are rare under the age of ten. We report a case of an incidentally colloid cyst presented in a 2.5 -years-old girl who was managed conservatively; follow-up with MRI showed a progressive reduction in size. We review the four similar case previously reported (AU)

Spontaneous resolution of colloid cyst of the third ventricle in a pediatric patient. / Regresión espontánea de quiste coloide de tercer ventrículo en paciente pediátrica.

Colloid cyst are benign intracranial lesions located in the III ventricle in the region of the foramen of Monro. The majority present in the third and fourth decades of life and are rare under the age of ten. We report a case of an incidentally colloid cyst presented in a 2.5 -years-old girl who was managed conservatively; follow-up with MRI showed a progressive reduction in size. We review the four similar case previously reported.

Tumor fibroso solitario/hemangiopericitoma de la médula espinal / Solitary fibrous tumour/haemangiopericytoma of the spinal cord

Los tumores fibrosos solitarios/hemangiopericitomas (TFS/HPC) son tumores mesenquimales fibroblásticos caracterizados por una fusión genética NAB2-STAT6 con una escasa incidencia en el sistema nervioso central (SNC), de los cuales los espinales son una minoría. Presentamos el caso de una mujer de 63 años con paraparesia progresiva y nivel sensitivo D6 que presentaba una lesión intradural extramedular a nivel D9-D10. Realizamos laminectomía con estudio ecográfico intraoperatorio en que visualizamos una lesión heterogénea con patrón infiltrativo afectando a la médula en varios puntos y realizamos resección de la misma. La immunohistoquímica reveló positividad para vimentina, CD34 y STAT6, Ki67 del 2%, diagnosticándose TFS/HPC fenotipo TFS. Los TFS/HPC del SNC son neoplasias raras que deberían tenerse en cuenta en el diagnóstico diferencial de este tipo de lesiones, la clínica y la resonancia magnética suelen llevar a errores en el enfoque diagnóstico. El apoyo de la ecografía intraoperatoria puede ayudar en la toma de decisiones quirúrgicas

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making

Solitary fibrous tumour/haemangiopericytoma of the spinal cord. / Tumor fibroso solitario/hemangiopericitoma de la médula espinal.

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.