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
Ependymoma tumors likely derive from the ependymal cells lining the CNS ventricular system. In grade II ependymomas, tumor cells resemble typical ependymocytes, while anaplastic ependymomas are poorly differentiated. We studied three grade II and one anaplastic ependymoma, focusing on the ciliary structures. To unambiguously characterize the ultrastructure and number of cilia, we performed electron microscopy serial section analysis of individual cells. Differentiated ependymomas contained large basal bodies and up to three cilia, and lacked centrioles. Anaplastic ependymoma cells showed instead two perpendicularly oriented centrioles and lacked cilia or basal bodies. These findings could contribute to understand the mechanisms of ependymoma aggressiveness.
Asunto(s)
Neoplasias Encefálicas/ultraestructura , Cilios/ultraestructura , Ependimoma/ultraestructura , Niño , Femenino , Humanos , Masculino , Microscopía Electrónica de Transmisión , Persona de Mediana Edad , Clasificación del Tumor , Adulto JovenRESUMEN
INTRODUCTION: Meningioma is the most common primary intracranial tumor, accounting for around a third of all primary brain tumor. In most cases, they are benign neoplasms that start in the cells of the arachnoid mater. Meningiomas are typically slow growing; however they can metastasize in 0.1% of all patients. CASE REPORT: A 45-years-old woman was admitted with a four weeks history of a painless enlarging mass in the right side of his neck and present in a MRI recurrence of the frontal meningioma surgically removed four times over the last 18 years. The patient underwent a complete removal of lesions and the microscopic observation showed an anaplastic meningioma in both (grade III of the World Health Organization). CONCLUSIONS: Metastatic meningioma is a rare cause of neck mass, but should be considered in any patient with a history of meningioma, especially if the tumor has histological features of malignant behavior. The present case demonstrates the aggressive biologic potential of an intracranial meningioma, with potential for distant spread and lymphatic invasion.
Asunto(s)
Neoplasias Meníngeas/patología , Meningioma/secundario , Resultado Fatal , Femenino , Humanos , Metástasis Linfática , Persona de Mediana Edad , CuelloRESUMEN
Introducción. Los meningiomas constituyen el tumor intracraneal más frecuente y representan una tercera parte de todos los tumores cerebrales primarios. En la mayoría de las ocasiones se trata de una neoplasia histológicamente benigna derivada de células aracnoideas, y en menos del 0,1% de los casos pueden presentar metástasis a distancia. Caso clínico. Mujer de 45 años que mostraba desde hacía cuatro semanas una tumoración dolorosa en la cara lateral derecha del cuello. En una resonancia magnética se objetivaba recidiva de un meningioma intracraneal intervenido en cuatro ocasiones en los últimos 18 años. Se realizó la extirpación del tumor intracraneal y de la masa cervical y el diagnóstico histológico de ambas lesiones demostró un meningioma anaplásico de grado III según la clasificación de la Organización Mundial de la Salud. Conclusiones. Las metástasis de los meningiomas son una causa poco frecuente de tumoración cervical; sin embargo, deben tenerse en consideración en pacientes que han sido intervenidos previamente de un meningioma que evidenciaba datos histológicos de malignidad. Nuestro caso demuestra el potencial agresivo que pueden tener ciertos meningiomas intracraneales con capacidad para diseminarse a distancia e invadir los ganglios linfáticos (AU)
Introduction. Meningioma is the most common primary intracranial tumor, accounting for around a third of all primary brain tumor. In most cases, they are benign neoplasms that start in the cells of the arachnoid mater. Meningiomas are typically slow growing; however they can metastasize in 0.1% of all patients. Case report. A 45-years-old woman was admitted with a four weeks history of a painless enlarging mass in the right side of his neck and present in a MRI recurrence of the frontal meningioma surgically removed four times over the last 18 years. The patient underwent a complete removal of lesions and the microscopic observation showed an anaplastic meningioma in both (grade III of the World Health Organization). Conclusions. Metastatic meningioma is a rare cause of neck mass, but should be considered in any patient with a history of meningioma, especially if the tumor has histological features of malignant behavior. The present case demonstrates the aggressive biologic potential of an intracranial meningioma, with potential for distant spread and lymphatic invasion (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Meningioma/complicaciones , Meningioma/diagnóstico , Meningioma/cirugía , Metástasis Linfática/fisiopatología , Metástasis Linfática , Meningitis Bacterianas/complicaciones , Meningitis Bacterianas/diagnóstico , Meningioma/fisiopatología , Meningioma , /métodos , /tendencias , Embolia Pulmonar/complicaciones , Embolia Pulmonar/mortalidad , Acinetobacter baumannii/aislamiento & purificaciónRESUMEN
INTRODUCTION: Neurocysticercosis, caused by the larvae of Taenia solium, is the most common parasitic infection of the central nervous system in humans. Considered an endemic parasitosis in developing countries including Latin America, Asia and Africa while in Europa, the cases of neurocysticercosis are anecdotal. CASE REPORTS: We report two cases of neurocysticercosis in children of non-Spanish origin who presented with seizures, with the initial diagnosis of brain tumors both were treated with surgery; later, to be the diagnosis of neurocisticercosis antiparasitic therapy was administered. CONCLUSIONS: Neurocysticercosis can be a potential cause of epilepsy even in non-endemic countries. Some cases may be difficult to diagnose and they can be confused with other intracranial lesions. Clinicians should be aware of this condition given increasing incidence in Spain and neurocysticercosis should be always be considered in the differential diagnosis particularly in patients from Latin America and Africa.
Asunto(s)
Neurocisticercosis , Niño , Preescolar , Femenino , Humanos , Masculino , Neurocisticercosis/diagnóstico , Neurocisticercosis/terapiaRESUMEN
Introducción. La neurocisticercosis es la enfermedad parasitaria más frecuente del sistema nervioso central y está causada por una infestación por la larva de la Taenia solium. Se trata de una enfermedad endémica en los países en vías de desarrollo, principalmente de América Latina, Asia y África, mientras que su presencia en Europa es anecdótica. Casos clínicos. Presentamos los casos de dos niños de origen no español, que comenzaron con una crisis epiléptica y que fueron diagnosticados inicialmente como tumores cerebrales primarios. Ambos fueron intervenidos quirúrgicamente y al diagnosticarse la parasitosis, recibieron posteriormente tratamiento antiparasitario. Conclusiones. La neurocisticercosis es una causa potencial de epilepsia incluso en aquellos países en los que no endémica. Numerosos casos presentan dificultades en el diagnóstico y se pueden confundir con otras lesiones intracraneales. Los facultativos debemos estar alerta, ya que se trata de una patología con un cierto repunte en España y se debe tener en cuenta en los diagnósticos diferenciales cuando tratamos pacientes procedentes de América Latina o África (AU)
Introduction. Neurocysticercosis, caused by the larvae of Taenia solium, is the most common parasitic infection of the central nervous system in humans. Considered an endemic parasitosis in developing countries including Latin America, Asia and Africa while in Europa, the cases of neurocysticercosis are anecdotal. Case reports. We report two cases of neurocysticercosis in children of non-Spanish origin who presented with seizures, with the initial diagnosis of brain tumors both were treated with surgery; later, to be the diagnosis of neurocisticercosis antiparasitic therapy was administered. Conclusions. Neurocysticercosis can be a potential cause of epilepsy even in non-endemic countries. Some cases may be difficult to diagnose and they can be confused with other intracranial lesions. Clinicians should be aware of this condition given increasing incidence in Spain and neurocysticercosis should be always be considered in the differential diagnosis particularly in patients from Latin America and Africa (AU)
Asunto(s)
Humanos , Masculino , Femenino , Niño , Neurocisticercosis/complicaciones , Neurocisticercosis/diagnóstico , Taenia solium/aislamiento & purificación , Epilepsia/complicaciones , Albendazol/uso terapéutico , Praziquantel/uso terapéutico , Imagen por Resonancia Magnética/métodos , Imagen por Resonancia Magnética , Neurocisticercosis/cirugía , Neurocisticercosis/tratamiento farmacológico , Neurocisticercosis/fisiopatología , Neurocisticercosis , Epilepsia/fisiopatología , Epilepsia , /métodos , /tendencias , Electroencefalografía/métodos , ElectroencefalografíaRESUMEN
INTRODUCTION: Primary bone lymphomas account for 3-7% of all malignant bone tumours and less than 2% of lymphomas in adults. Having the cranial vault as their primary location is very infrequent. CASE REPORT: A 59-year-old female who presented a painful tumour on the cranial vault which, following radical surgical removal, was found to be an ALK-negative primary large-cell lymphoma. CONCLUSIONS: After reviewing the literature we believe that this is the first case of cranial vault lymphoma in which the translocation of the ALK protein-producing gene has been studied.
Asunto(s)
Linfoma de Células B Grandes Difuso/patología , Neoplasias Craneales/patología , Cráneo/patología , Quinasa de Linfoma Anaplásico , Femenino , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/genética , Linfoma de Células B Grandes Difuso/cirugía , Persona de Mediana Edad , Proteínas Tirosina Quinasas Receptoras/genética , Proteínas Tirosina Quinasas Receptoras/metabolismo , Neoplasias Craneales/diagnóstico , Neoplasias Craneales/genética , Neoplasias Craneales/cirugía , Tomografía Computarizada por Rayos XRESUMEN
Introducción. Los linfomas primarios del hueso representan el 3-7% de todos los tumores óseos malignos de los huesos y menos del 2% de todos los linfomas del adulto. Su localización primaria en la bóveda craneal es excepcional. Caso clínico. Mujer de 59 años que presentó una tumoración dolorosa sobre la calota craneal y que tras la extirpación quirúrgica radical se comprobó que se trataba de un linfoma primario de células grandes ALK negativo. Conclusión. Tras la revisión de la bibliografía consideramos que se trata del primer caso de linfoma de calota craneal en el que se estudió la traslocación del gen productor de la proteína ALK (AU)
Introduction. Primary bone lymphomas account for 3-7% of all malignant bone tumours and less than 2% of lymphomas in adults. Having the cranial vault as their primary location is very infrequent. Case report. A 59-year-old female who presented a painful tumour on the cranial vault which, following radical surgical removal, was found to be an ALK-negative primary large-cell lymphoma. Conclusions. After reviewing the literature we believe that this is the first case of cranial vault lymphoma in which the translocation of the ALK protein-producing gene has been studied (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Neoplasias Craneales/patología , Linfoma Anaplásico de Células Grandes/patología , Neoplasias Óseas/patología , Tomografía Computarizada por Rayos X , Craneotomía/métodosRESUMEN
Solid cell nests (SCNs) of the thyroid are single or multiple foci of solid and/or cystic clusters of squamoid cells (main cells) with a minor proportion of C-cells, found in the normal thyroid. The SCNs have also been reported in the heart as an ultimobranchial heterotopia. Here, the authors describe a case of thyroid-type SCNs associated with struma ovarii. Main cells were positive for simple and stratified epithelial-type cytokeratins, carcinoembryonic antigen, carbohydrate antigen 19.9, p63, bcl-2, and galectin-3. The neuroendocrine cell population was positive for chromogranin A and synaptophysin but negative for calcitonin, suggesting a common ancestor cell capable of dual differentiation toward thyroid follicular cells and hindgut-type endocrine cells. The existence of thyroid-type SCNs in struma ovarii could be easily understood by considering the struma ovarii as a teratoma; at the same time, these findings also support the idea of a close histogenetic link between the main cells of SCNs and thyroid tissue.
Asunto(s)
Neoplasias Ováricas/patología , Estruma Ovárico/patología , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Neoplasias Ováricas/metabolismo , Estruma Ovárico/metabolismoRESUMEN
Solid cell nests of the human thyroid gland are composed of main cells and C cells. In order to investigate the putative stem cell nature of the role for solid cell nests, we evaluated the histological features, and the immunohistochemical expression of p63, bcl-2, telomerase catalytic subunit, and two proliferative markers (Ki-67 and minichromosome maintenance protein 2), in a series of 24 cases of solid cell nests. Proliferative indices were determined in (a) solid cell nests, (b) thyroid follicular cells in the vicinity of solid cell nests within a low-power field, and (c) distant thyroid tissue, at a distance of at least three low-power fields from solid cell nests. In 15 cases of solid cell nests (62.5%), mixed follicles were observed; papillary formations were observed in four cases (16.6%), and ciliated cells were observed in the lining of microcysts associated with two cases (8.3%). Salivary gland-type tissue, cartilage islands, adipose and fibrous tissues, and small nerves were also associated with some cases of solid cell nests. We observed that the main cells of the solid cell nests express consistently telomerase, although at lower levels than p63, and show strong cytoplasmic immunoreactivity for bcl-2, which is associated with an increased differentiation potential. We also observed that despite their relative low proliferative index, main cells of the solid cell nests display higher proliferation than follicular cells in the vicinity and follicular cells in more distant thyroid tissue. We conclude that main cells of the solid cell nests apparently harbor the minimal properties of a stem cell phenotype (capacity for both self-renewal, conferred by telomerase activity, and differentiation to one or more than one type of specialized cells, given by the high expression of p63 and bcl-2) and may thus represent a pool of stem cells of the adult thyroid.
Asunto(s)
Telomerasa/biosíntesis , Glándula Tiroides/química , Adulto , Anciano , Anciano de 80 o más Años , Proteínas de Ciclo Celular/análisis , Diferenciación Celular , División Celular , Proteínas de Unión al ADN , Femenino , Genes Supresores de Tumor , Humanos , Inmunohistoquímica , Antígeno Ki-67/análisis , Masculino , Persona de Mediana Edad , Componente 2 del Complejo de Mantenimiento de Minicromosoma , Proteínas Nucleares/análisis , Fosfoproteínas/análisis , Proteínas Proto-Oncogénicas c-bcl-2/análisis , Células Madre/química , Células Madre/fisiología , Glándula Tiroides/patología , Glándula Tiroides/fisiopatología , Transactivadores/análisis , Factores de Transcripción , Proteínas Supresoras de TumorRESUMEN
Mesenchymal hamartoma (MH) is a rare liver lesion of infancy. Due to its rapid increase in size, it is often misdiagnosed clinically as a malignant tumour or as a hepatic cyst because of its cystic appearance. We present the clinicopathological, immunohistochemical and flow cytometric features of two cases, involving an 11-month-old boy and a 13-month-old girl. In both cases, the histological appearance and the immunohistochemical findings were identical. Bile ducts and blood vessels showed the expected immunohistochemical profile, whereas the mesenchymal component showed immunoreactivity not only for vimentin but also for muscular markers. Flow cytometry disclosed an aneuploid population in one case, thus favouring the interpretation of MH as a neoplastic lesion. Unlike the characteristically continuous and rapid growth of MH before or shortly after birth, these two cases showed low proliferative and apoptotic indexes and a high immunohistochemical expression of bcl-2 protein. This prompted us to hypothesize that MH might undergo a brief initial proliferative phase, but the cells would later become 'immortalized' by bcl-2 overexpression.
RESUMEN
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.