RESUMEN
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
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Médula Espinal/diagnóstico por imagen , Hemangiopericitoma/diagnóstico por imagen , Hemangiopericitoma/cirugía , Médula Espinal/patología , Espectroscopía de Resonancia Magnética , InmunohistoquímicaRESUMEN
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.
Asunto(s)
Hemangiopericitoma/cirugía , Tumores Fibrosos Solitarios/cirugía , Neoplasias de la Médula Espinal/cirugía , Biomarcadores de Tumor/análisis , Descompresión Quirúrgica , Errores Diagnósticos , Femenino , Hemangiopericitoma/química , Hemangiopericitoma/complicaciones , Hemangiopericitoma/diagnóstico por imagen , Humanos , Laminectomía , Vértebras Lumbares/cirugía , Imagen por Resonancia Magnética , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Persona de Mediana Edad , Neuroimagen , Paraplejía/etiología , Tumores Fibrosos Solitarios/química , Tumores Fibrosos Solitarios/complicaciones , Tumores Fibrosos Solitarios/diagnóstico por imagen , Trastornos Somatosensoriales/etiología , Neoplasias de la Médula Espinal/química , Neoplasias de la Médula Espinal/complicaciones , Neoplasias de la Médula Espinal/diagnóstico por imagen , Fusión Vertebral , Estenosis Espinal/cirugía , Cirugía Asistida por Computador , Vértebras Torácicas , Ultrasonografía IntervencionalRESUMEN
La radiocirugía estereotáctica constituye sin lugar a dudas un tratamiento de primer orden en una gran variedad de procesos intracraneales, especialmente de naturaleza tumoral, y no está exenta de complicaciones. Actualmente se considera un buen tratamiento para una gran mayoría de schwannomas del nervio vestibular de pequeño y mediano tamaño. Los autores describimos el caso clínico de una mujer de 69 años que desarrolló un glioma de alto grado secundariamente al tratamiento radioquirúrgico de un schwannoma del nervio vestibular. Describimos las características de nuestro caso y realizamos una revisión de la literatura
Stereotactic radiosurgery undoubtedly represents an important therapeutic procedure for various intracranial pathologies, especially tumours, although they are not entirely complication-free. Radiosurgery have been considered a good management strategy for the majority of small-to-medium size vestibular schwannomas. The authors describe a case of high-grade glioma associated with a previous radiosurgery treatment for a vestibular schwannoma in a 69 year-old woman. A detailed description of these cases is provided, as well as a summary of the related literature
Asunto(s)
Humanos , Femenino , Anciano , Glioma/etiología , Neoplasias Inducidas por Radiación/diagnóstico , Radiocirugia/efectos adversos , Neuroma Acústico/cirugía , Complicaciones PosoperatoriasRESUMEN
Stereotactic radiosurgery undoubtedly represents an important therapeutic procedure for various intracranial pathologies, especially tumours, although they are not entirely complication-free. Radiosurgery have been considered a good management strategy for the majority of small-to-medium size vestibular schwannomas. The authors describe a case of high-grade glioma associated with a previous radiosurgery treatment for a vestibular schwannoma in a 69 year-old woman. A detailed description of these cases is provided, as well as a summary of the related literature.
Asunto(s)
Neoplasias Encefálicas/etiología , Glioma/etiología , Neoplasias Inducidas por Radiación/etiología , Neuroma Acústico/cirugía , Radiocirugia/efectos adversos , Lóbulo Temporal , Anciano , Neoplasias Encefálicas/patología , Femenino , Glioma/patología , Humanos , Clasificación del Tumor , Neoplasias Inducidas por Radiación/patologíaRESUMEN
INTRODUCTION: Angiolipoma is a benign tumour composed of mature adipocytes admixed with abnormal vascular elements. It reportedly accounts for 0.1-0.5% of all spinal axis tumours in adults and is extremely rare in children. CASE REPORT: This article describes a case of thoracic spinal angiolipoma in a 4-year-old boy, who presented with back pain and weakness. The tumour was excised, and the child made a remarkable recovery. DISCUSSION: Only five previous cases of extradural angiolipoma have been reported in children. The authors review the existing literature on the aetiology, pathogenesis, and clinical pathology, and the surgical management is discussed.