Correlación entre aspectos clínicos, imagenológicos e histopatológicos en el diagnóstico de tumores y seudotumores raquídeos extramedulares / Correlation between clinical, imaging, histological and pathological aspects in the diagnosis of spinal extramedular tumors and pseudotumors i: en

Introducción: Los tumores intradurales extramedulares son causa de morbilidad y mortalidad. Para identificarlos y analizarlos, la resonancia magnética resulta el método diagnóstico imagenológico de elección. Objetivo: Caracterizar a pacientes con tumores y seudotumores raquídeos intradurales extramedulares según variables clínicas, imagenológicas (por resonancia magnética) e histopatológicas. Métodos: Se efectuó un estudio observacional, descriptivo, longitudinal y prospectivo en 20 pacientes con diagnóstico clínico de sospecha de tumor o seudotumor intradural extramedular o no, confirmado mediante resonancia magnética, quienes fueron atendidos en el Departamento de Imagenología del Hospital General Docente Dr. Juan Bruno Zayas Alfonso de Santiago de Cuba de enero del 2014 a igual mes del 2017, procedentes de los hospitales generales de esta provincia y de Guantánamo. Resultados: Existió una mayor frecuencia del sexo masculino (70,0 %), principalmente en las edades de 60 y más años (30,0 %), así como del dolor vertebral (100,0 %) y las alteraciones de los reflejos osteotendinosos (65,0 %) como síntomas y signos de los procesos neoplásicos. Según la intensidad de las señales captadas en la resonancia magnética, primaron los tumores isointensos en la secuencia T1 (70,0 %) y los hiperintensos e isointensos en la secuencia T2 (35,0 % en cada uno), sobre todo de localización dorsal (60,0 %). Asimismo, el diagnóstico clínico de sospecha más usual fue el de mielopatía compresiva (25,0 %), en tanto, entre los diagnósticos imagenológicos e histopatológicos sobresalieron las metástasis y los meningiomas. Conclusiones: La resonancia magnética ofrece una aproximación al diagnóstico definitivo de estos tumores, el cual es corroborado con el estudio histopatológico.

Introduction: The intradural extraspinal tumors are morbidity and mortality cause. To identify and analyze them, the magnetic resonance is the imaging diagnostic means of election. Objective: To characterize patients with intradural extramedular spinal tumors and pseudotumors according to clinical, imaging (by magnetic resonance) histological and pathological variables. Methods: An observational, descriptive, longitudinal and prospective study was carried out in 20 patients with suspicious clinical diagnosis of intradural extraspinal tumor or pseudotumor or not, confirmed by means of magnetic resonance who were assisted in the Imaging Department of Dr. Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba from January, 2014 to the same month in 2017, coming from the general hospitals of this province and Guantánamo. Results: There was a higher frequency of the male sex (70.0 %), mainly 60 and more years (30.0 %), as well as of the vertebral pain (100.0 %) and disorders of the muscle stretch reflex (65.0 %) as symptoms and signs of the neoplasm processes. According to the intensity of the signs captured in the magnetic resonance, the isointense tumors prevailed in the sequence T1 (70,0 %) and the hyperintense and isointense in the sequence T2 (35.0 % in each one), mainly of dorsal localization (60.0 %). Also, the most common supicious clinical diagnosis was that of compressive myelopathy (25.0 %), as long as, among the imaging, histological and pathological diagnosis the metastasis and meningiomas were notable. Conclusions: The magnetic resonance offers an approach to the definitive diagnosis of these tumors, which is corroborated by means of the histological and pathological study.

Solitary Dorsal Intramedullary Schwanomma ­ A Rare Lesion

Intramedullary schwanommas are rare, and most cases are reported in cervical region. Less than 20 dorsal intramedullary schwanommas have been reported till date in literature. This is due to their cell of origin, the Schwann cell, which is not normally found within the parenchyma of the brain and spinal cord; therefore it is not surprising that these lesions are rare. We report a rare solitary dorsal intramedullary schwanomma in a young adult patient who presented with paraplegia.

Cystic paraganglioma of the filum terminale / Paraganglioma quístico del filum terminal

Spinal Paragangliomas are rare tumors, most frequently involving the cauda equina and the filum terminale. We report the case of a 62-year old woman presenting with a one month's lower back pain, left sciatica and leg weakness. Spinal magnetic resonance imaging showed a extramedullary lesion at L3-L4 level, measuring 37 x 52 x 21 mm with contrast enhancement. It presented an unenhancing intradural cystic lesion in the upper pole. The patient underwent lumbar laminectomy, and with neurofisiologic monitorization the tumor was completely resected. Microscopic examination of the tumor revealed a paraganglioma. In a review of the literature, we found only five previous reported cases in which the tumor is accompanied by a cyst.

Los paragangliomas son tumores poco frecuentes que se localizan en la región de la cauda equina y filum terminal. Presentamos el caso de una mujer de 62 años con una historia de 1 mes de evolución de lumbalgia, ciática y debilidad en el miembro inferior izquierdo. Una resonancia magnética lumbar mostró una tumoración extramedular en el nivel L3-L4 que medía 37 x 52 x 21 mm que se realzaba con contraste. Presentaba así mismo una lesión quística en el polo superior del tumor. Se realizó una laminectomía lumbar y bajo control neurofisiológico se realizó una extirpación completa el tumor. El estudio microscópico revelo que se trataba de un paraganglioma. Realizamos una revisión de la literatura encontrando únicamente otros cinco casos de paraganglioma espinal con lesión quística acompañante

Intradural Spinal Arachnoid Cyst in a Pediatric Patient: A Case Report

The present case reports a 13-year-old patient with an intradural arachnoid cyst, which manifested itself with a sudden loss of strength and sensitivity in the lower and upper limbs and a severe pain in the cervical and thoracic region. On examination, a lesion displayed as an intradural hematoma; however, a laminotomy was performed and it was realized that the lesion was an arachnoid spinal cyst of the cervical-dorsal spine.

Tumores Intramedulares. Experiencia reciente / intramedullary tumors. recent experience

Introducción: Los tumores intramedulares (TIM) constituyen una patología infrecuente siendo pasibles de resección radical con alto riesgo de deterioro neurológico definitivo. Objetivos: Evaluar factores determinantes de la evolución tales como la técnica quirúrgica empleada, la utilidad del monitoreo neurofisiológico y la evolución de los casos tratados recientemente en nuestro Servicio. Material y Métodos: Revisamos retrospectivamente las Historias Clínicas de pacientes diagnosticados como tumores espinales entre diciembre de 2011 y diciembre 2018. Se consigna el examen neurológico pre y post operatorio, extensión tumoral, descripción de la técnica quirúrgica y los registros obtenidos durante el monitoreo neurofisiológico. Se reflejó la evolución en la Escala de McCormick inmediata, a 6 y 12 meses. Las IRM de ingreso y posoperatoria fueron comparadas para determinar el grado de resección. Se registraron los procedimientos de estabilización ortopédica, así como el tratamiento oncológico complementario. Resultados: Sobre un total de 51 Tumores espinales registrados en el período estudiado, 13 de ellos fueron diagnosticados como TIM. 7 Femeninas, 6 Masculinos. Edad media: 8 años (8 meses a 14 años). Seguimiento de 6 meses a 5 años. Sintomatología: Trastorno de la marcha: 5, Dolor: 5, Escoliosis: 4, Trastornos vesicales: 3, Tortícolis: 2.Características en neuroimágenes: Sólido 8, Sólido/quístico 5, Siringomielia asociada: 5 Técnica quirúrgica: Laminoplastía: 12 pacientes. Laminectomía: 1. Monitoreo intraoperatorio: Atenuación de la onda menor 50%: 5, atenuación mayor 50%: 6.Resección Total: 7, Subtotal/Parcial: 4, Biopsia: 2. Reoperación: una paciente portadora de Ependimoma con residuo en cara anterior medular.Resultado postoperatorio al año: Empeorado: 3. Sin cambios: 5. Mejoría: 5. No se registraron fallecimientos relacionados con el procedimiento.Quimioterapia adyuvante: 2. El déficit funcional mediato mejoró o se mantuvo estable en 12 casos. Se obtuvo recuperación a los 6 meses en 11 pacientes. Un paciente que adicionalmente sufrió un empiema a la semana no recuperó la paraplejía al año de la cirugía. Otro paciente falleció en el curso de la quimioterapia en los dos meses posoperatorios. Conclusiones: El empeoramiento en la función neurológica es esperable en el posoperatorio inmediato. La mayoría de los pacientes exhiben recuperación en los meses siguientes. La resección radical es factible y permite curación o favorece el tratamiento oncológico a largo plazo acorde a la biología tumoral.

Introduction: Intramedullary tumors constitute an infrequent pathology, being capable of radical resection with a high risk of definitive neurological deterioration. Objectives: Evaluate outcome determinants such as the surgical technique used, the usefulness of neurophysiological monitoring and the evolution of the cases recently treated in our Hospital. Material and Methods: We retrospectively reviewed the clinical records of patients diagnosed as spinal tumors between December 2011 and December 2018. The pre and post-operative neurological examination, tumor extension, description of the surgical technique and the information obtained during neurophysiological monitoring are recorded. The evolution was analyzed by the McCormick Scale, at 6 and 12 months. Admission and postoperative MRIs were compared to determine the degree of resection. Orthopedic stabilization procedures as well as complementary oncological treatment were studied. Results: Follow up: 6 months to 5 years. 7 Female, 6 Male. Average age: 8 years (8 months to 14 years) Symptoms: Gait disturbance: 5, Pain: 5, Scoliosis: 4, Bladder Disturbance: 3, Tortícolis: 2.MRI: Solid 8, Solid/ cystic 5, associated Syrinx: 5Surgery: Laminoplasty: 11. Laminectomy: 2. Surgery monitoring : flattened wave less to 50%: 5, up to 50%: 6.Gross total removal: 7, Subtotal/Partial: 4, Biopsy: 2. New exploration: a patient with anterior spinal cord remained Ependymoma.Surgical results: Worsened : 3. Unchanged: 5. Improvement: 5Chemotherapy : 2. Improvement of functional deficit was seen in 11 cases between 6-12 months. There were no deaths due the surgery. Conclusions: Worsening of neurological function is usual immediately to intramedullary surgery. Mostly of patients will have a recovery. Radical resection of this tumors is feasible and in some cases curative, in other cases provide better chemotherapy opportunities.

Cervical Osteoblastoma: A Case Report / Osteoblastoma cervical: relato de caso

Abstract Osteoblastoma is a rare benign primary bone tumor. It accounts for 3% of benign and 1% of all primary bone tumors. The treatment goal is complete surgical resection. This treatment limits the risk of recurrence. As osteoblastoma is a highly vascular tumor, complete resection is often difficult. This report describes the case of a 19-year-old male patient who presented severe right-sided neck and shoulder pain. The computed tomography scan revealed a mass lesion on C7 compatible with osteoblastoma. Preoperative embolization and tumor resection were performed. At the 3-year follow-up, the patient had no restrictions on daily activities, and, to date, there has been no evidence of recurrence.

Resumo Osteoblastoma é um tumor primário benigno raro. Constitui cerca de 3% dos tumores benignos e 1% de todos os tumores ósseos. O objetivo do tratamento é a ressecção completa, que limita o risco de recidiva. Por se tratar de um tumor vascularizado, a ressecção completa é, muitas vezes, difícil. Os autores descrevem um caso clínico de um paciente do sexomasculino de 19 anos que apresentava queixas de cervicalgia direita com irradiação para o ombro. O exame por tomografia computadorizada indicou uma lesão em C7 compatível com osteoblastoma. O paciente foi submetido a resseção cirúrgica após embolização pré-operatória e artrodese anterior. Aos 3 anos de seguimento, o paciente encontra-se assintomático e, até a data, sem evidência de recidiva.

Intradural Extramedullary Spinal Metastasis of a Kidney Cancer: Case Report

Objective To report a case of intradural extramedullary metastasis originated from a kidney neoplasm. Metastases in this topography occur in a low frequency, especially considering that the primary tumor was located along the urinary system. Case Report Amale adult begins to present with pain in the lower limbs and develops flaccid paraparesis of the pelvic limbs, also attacking the control of the sphincter. The patient had a previous history of right nephrectomy due to a kidney neoplasm. After investigation with imaging exams, ametastatic lesion was found to be the source of the symptoms. The patient was surgically treated by the neurosurgery team of the hospital. Conclusion Cases like this are not common, and considering the low incidence of these cases and the nonspecific symptoms, such as pain, we do not always come up with the hypothesis of a metastasis in this topography. The surgical treatment, although it is a palliative feature, has an important part inmaintaining the performance and the quality of life of the patient.

Caracterización clínica epidemiológica de pacientes operados de tumores raquimedulares / Clinical epidemiological characterization of patients operated on spinal cord tumors

Introducción: Los tumores raquimedulares son la mayor causa de morbilidad y mortalidad entre pacientes con afecciones oncológicas y representan aproximadamente del 10 al 15 por ciento de todos los tumores del sistema nervioso central. Objetivo: Identificar aspectos clínicos y epidemiológicos en pacientes operados de tumores raquimedulares. Métodos: Se realizó un estudio observacional descriptivo, de serie de casos, en 31 pacientes de más de 19 años, operados/as de tumores intrarraquídeos, en el servicio de Neurocirugía del Hospital Provincial Docente "Saturnino Lora" de Santiago de Cuba, durante el período comprendido desde enero del 2008 a diciembre del 2013. Resultados: En los casos estudiados (n= 31) la edad tuvo un recorrido entre 19 y 76 años, predominó el sexo femenino (54,8 por ciento), las lesiones benignas (64,5 por ciento), así como mayor localización dorsal y extradural en el eje espinal. Conclusiones: Las principales características clínicas y epidemiológicas fueron: edad de presentación más frecuente en la sexta década de la vida, sintomatología con compromiso neurológico variable, dolor del raquis en todos los casos y predominio de lesiones benignas(AU)

Introduction: Spinal cord tumors are the leading cause of morbidity and mortality among patients with oncological conditions and it represents approximately 10 percent to 15 percent of all tumors of the central nervous system. Objective: To identify clinical and epidemiological aspects of patients operated on spinal cord tumors, from 2008 to 2013. Methods: We conducted an observational, descriptive study of a series of cases in 31 patients older than 19 years at the Neurosurgery Service of Saturnino Lora Provincial Teaching Hospital in Santiago de Cuba from January 2008 to December 2013. The subjects had diagnosis of intratracheal tumors. Results: In the cases studied (n = 31) age ranged 19 to 76 years. Female patients (54.8percent) and benign lesions (64.5 percent) were the majority. The most frequent localization was dorsal and extradural in the spinal axis. Conclusions: The main clinical and epidemiological characteristics were age of more frequent presentation of tumor lesions, that is 60 years of age; symptomatology with variable neurological involvement, spinal pain in all cases, and predominance of benign lesions(AU)

Extradural spinal cord hemangioblastoma: a case report and literature review / Hemangioblastoma extradural medular: relato de caso e revisão de literatura / Hemangioblastoma extradural de la médula espinal: informe de un caso y revisión de la literatura

ABSTRACT Objective: Hemangioblastoma is a rare vascular sporadically occurring CNS tumor that can be associated with von Hippel-Lindau disease. Hemangioblastomas account for 2-6% of all spinal cord neoplasms and rank third among intramedullary space-occupying lesions. Methods: This was the first time in our practice that we had dealt with paravertebral hemangioblastoma with the sandglass growth pattern. The world literature describes only 3 case of a tumor with this growth pattern. Surgical and diagnostic aspects of patient treatment are considered. Results: During the operation, we adhered to the following stages: localization of the feeding vessel and of the poles of the tumor, surface dissection of the tumor, en bloc resection of the tumor, and hemostasis of the tumor cavity. Conclusions: Hemangioblastoma of extradural localization is a very rare pathology. However, when MRI signs characteristic of a vascular lesion are identified, it is necessary to carry out additional examinations, which may include CT perfusion study and, if required, selective angiography. Level of Evidence 5; Case report.

RESUMO Objetivos: Hemangioblastoma é um tumor vascular raro do SNC, que ocorre esporadicamente e pode ser associada à doença de Von Hippel-Lindau. Hemangioblastomas são responsáveis por 2-6% de todas as neoplasias da medula espinal e na terceira posição entre lesões, ocupando espaço intramedulares da medula espinal. Métodos: Foi a primeira vez em nossa prática que lidamos com hemangioblastoma paravertebral com o padrão de crescimento ampulheta. As fontes da literatura mundial descrevem apenas 3 casos de um tumor com esse padrão de crescimento. Aspectos cirúrgicos e diagnósticos de tratamentos do paciente são considerados. Resultados: Durante a operação, aderiu-se às seguintes fases: o recipiente de alimentação foi encontrado, os pólos do tumor foram encontrados, a dissecação da superfície do tumor foi feita, o tumor foi removido por um único bloco e a hemostasia da cavidade do tumor foi realizada. Conclusões: Hemangioblastoma de localização extradural é uma patologia muito rara, no entanto, quando sinais de MRI característicos de uma lesão vascular são identificados, o que é necessário para levar a cabo um exame suplementar, o qual pode incluir o estudo de perfusão CT e, se necessário, angiografia seletiva. Nível de Evidência V; Relato de caso.

RESUMEN Objetivo: El hemangioblastoma es un tumor vascular raro del SNC que ocurre esporádicamente y que puede asociarse con la enfermedad de von Hippel-Lindau. Los hemangioblastomas representan el 2%-6% de todas las neoplasias de la médula espinal y están en el tercer lugar entre las lesiones intramedulares que ocupan espacio. Métodos: Esta fue la primera vez en nuestra práctica que tratamos el hemangioblastoma paravertebral con patrón de crecimiento de reloj de arena. La literatura mundial describe solo tres casos de un tumor con este patrón de crecimiento. Se consideran aspectos quirúrgicos y de diagnóstico del tratamiento del paciente. Resultados: Durante la operación, elegimos las siguientes etapas: localización del vaso de irrigación y de los polos del tumor, disección superficial del tumor resección en bloque del tumor y hemostasia de la cavidad tumoral. Conclusiones: El hemangioblastoma de localización extradural es una patología muy rara. Sin embargo, cuando se identifican signos característicos de lesión vascular en la RM, es necesario realizar exámenes adicionales, que pueden incluir estudio de perfusión por TC y, si es necesario, una angiografía selectiva. Nivel de Evidencia V; Reporte de caso.

Mielitis Transversa Análisis Clínico y Revisión de Caso / Transverse Myelitis, Clinical Analysis and Case Review

Resumen: La mielitis transversa es una enfermedad inflamatoria y desmielinizante aguda o subaguda que se desarrolla en ausencia de afectación neurológica previa y compromete las vías sensitiva y motora además del control autónomo de la medula espinal. El cuadro se presenta como un dolor lumbar localizado, parestesias de inicio súbito en miembros inferiores con pérdida de la sensibilidad y paraparesia que puede evolucionar a paraplejia a lo que se suma comúnmente la disfunción vesical e intestinal; la coordinación y la sensibilidad de los miembros superiores también puede verse comprometida La incidencia de esta patología es baja, en los Estados Unidos oscila entre 14.000 casos nuevos en el año, de los cuales 33.000 persisten con secuelas. Afecta a hombres, mujeres y niños de todas las razas por igual, presentándose con más frecuencia entre los 10-19 años y los 30-39 años de edad. El diagnóstico diferencial incluye: síndrome de Guillain-Barré, compresión medular por tumores, mielopatías de origen vascular, esclerosis múltiple, neuromielitis óptica entre otros. El diagnóstico se basa en la presunción clínica, ante la cual debe solicitarse una resonancia magnética de manera urgente. El siguiente paso es realizar una punción lumbar para estudio en líquido cefalorraaquídeo (LCR) de células blancas, IgG y albúmina. El tratamiento de la mielitis transversa va encaminado hacia la resolución del proceso inflamatorio a nivel medular y la detención del avance del mismo.

Abstract Transverse myelitis is an acute or subacute inflammatory demyelinating disease that develops in the absence of previous neurological involvement and compromises the sensory and motor pathways in addition to the autonomous control of the spinal cord, the table is presented as a localized back pain, sudden paresthesia in lower limbs with loss of sensation and paraparesis which may progress to paraplegia commonly associated with bladder and bowel dysfunction; coordination and sensitivity of the upper limbs may also be compromised The incidence of this disease is low, in the United States ranges from 14,000 new cases per year of which 33,000 remain with sequelae. It affects men, women and children of all races equally predominantly between 10-19 years and 30-39 years of age. Differential diagnoses include Guillain- Barré syndrome, compression by spinal cord tumors, vascular myelopathy, multiple sclerosis, neuromyelitis óptica spectrum diseases amongst others. Diagnosis is based on clinical suspicion upon which an MRI should be requested urgently, the next step is to perform a lumbar puncture to study CSF white cell count, IgG and albumin. The treatment of transverse myelitis is aimed towards resolution of the inflammatory process in the spinal cord and avoiding neural deficit progression.

Resultados neurológicos en pacientes con diagnóstico de tumores raquídeos intradurales extramedulares / Neurological results in patients diagnosed with intradural spinal tumors

Se presentan a continuación los resultados neurológicos de 10 pacientes con diagnóstico de tumor intramedular extradural que fueron intervenidos quirúrgicamente en el Hospital Teodoro Maldonado Carbo durante el periodo enero 2015 a junio 2017. Se encontraron ocho hombres (80 %) y dos mujeres (20 %) con un promedio de edad de 44,4 años. El periodo de seguimiento posoperatorio fue de seis meses. La clínica neurológica,localización axial y sagital de los tumores, resultados histopatológicos fueron analizados. Los resultados neurológicos encontrados en el preoperatorio y en el periodo posoperatorio a los seis meses de seguimiento fueron evaluados de acuerdo con la clasificación ASIA (American Spinal Injury Association); y, el Score de Rankin modificado. Los resultados histopatológicos fueron los siguientes: cuatro casos (40 %) de schwannoma, tres casos (30 %) de meningioma, un caso (10 %) de plasmocitoma, un caso (10 %) de ependimoma y un caso (10 %) de metástasis de otros tumores. Se presentaron complicaciones en el 20 % de casos: un caso (10 %) de infección de herida quirúrgica; y, otro caso (10 %) de fístula de líquido cefalorraquídeo. En el 70 % de casos, al seguimiento seis meses luego de la cirugía se encontró mejoría en la clínica neurológica; y, en 30 % de los casos no hubo cambios neurológicos. Al valorar la Escala de Rankin modificada se encontró que tres pacientes (30 %) se recuperaron completamente, evolucionando desde un grado 2 (incapacidad leve) a un grado 1 (sin incapacidad); por otro lado, cuatro casos (40 %) mejoraron su grado de incapacidad funcional; y, tres casos (30 %) no mostraron cambios en cuanto a suincapacidad funcional, es decir, sin variaciones en su clínica neurológica. Podemos concluir que el diagnóstico oportuno y el tratamiento precoz mejoran la morbilidad y el grado de discapacidad, puesto que, ante un mayor déficit neurológico menor es el resultado pronóstico neurológico. Los resultados dependerán, por lo tanto, del momento oportuno de la intervención quirúrgica raquídea descompresiva.

We present the neurological results of 10 patients diagnosed with Extradural Intramedullary Tumor who underwent surgery at Teodoro Maldonado Carbo Hospital during the period January 2015 to June 2017. Eight men (80 %) and two women (20 %) were found with an average age of 44,4 years. The postoperative follow-up period was six months. Neurological symptoms, axial and sagittal localization of the tumors, histopathological results were analyzed. The neurological results found in the preoperative period and in the postoperative period at six months of follow-up were evaluated according to the ASIA (American Spinal Injury Association) classification; and, the modified Rankin Score. The histopathological results were the following: four cases (40 %) of schwannoma, three cases (30 %) of meningioma, one case (10 %) of plasmacytoma, one case (10 %) of ependymoma and one case (10 %) of metastasis of other tumors. Complications occurred in 20 % of cases: one case (10 %) surgical wound infection, and in anothercase (10 %) cerebrospinal fluid fistula. In 70 % of cases, at follow-up six months after surgery, improvement was found in the neurological clinic; and, in 30 % of the cases there were not neurological changes. When assessing the modified Rankin scale, it was found that three patients (30 %) recovered completely, evolving from a grade 2 (mild disability) to a grade 1 (without disability); On the other hand, four cases (40 %) improved their degree of functional disability; and, three cases (30 %) did not show changes in their functional disability, that is, without variations in their neurological clinic. We can conclude that timely diagnosis and early treatment improves morbidity and the degree of disability, since, in the face of a greater neurological deficit, the neurological outcome is lower. The results will therefore depend on the opportune moment of the decompression spinal surgery.

Neurofibroma cervicodorsal en una niña con neurofibromatosis tipo 1 / Cervical dorsal neurofibroma in a girl with neurofibromatosis type 1 neurofibroma / Neurofibroma cervicodorsal em uma criança com neurofibromatose tipo 1

La neurofibromatosis es el síndrome neurocutáneo más frecuente, una enfermedad hereditaria con afectación multisistémica. Se distinguen tres formas clínicas de la enfermedad; la neurofibromatosis tipo 1 (NF-1) es la más frecuente. A las lesiones cutáneas, presentes en la mayoría de los casos, se pueden asociar tumores en cualquier localización. Los neurofibromas son característicos de la NF-1. El objetivo de esta comunicación es describir una complicación poco frecuente de la NF-1 que representa un desafío terapéutico debido a la topografía y frecuencia de recidivas. Se trata de una niña de 3 años con NF-1 con síntomas de mielopatía multisegmentaria de instalación progresiva de dos meses de evolución. La imagenología evidenció una tumoración cérvico-dorsal con compresión medular. La exéresis tumoral permitió confirmar neurofibroma intradural, extramedular. Los neurofibromas plexiformes se caracterizan por su comportamiento infiltrativo y diseminación paraespinal extensa. La resección de estas lesiones genera habitualmente morbilidad asociada al compromiso de estructuras críticas extraespinales y dificulta su resección completa tal como ocurrió en el caso clínico presentado. Se destaca la importancia de realizar un correcto seguimiento clínico y eventualmente imagenológico de los pacientes con NF-1 para detectar y abordar oportunamente los tumores, su complicación más importante.

Neurofibromatosis is one of the most frequent neurocutaneous syndrome. It is an inherited diseasewhich affects multiple systems. Three clinical presentations of the disease can be distinguished being type 1 neurofibromatosis the most common. Tumors at any body location can be associated to the usually presented cutaneous lesions. Neurofibromas are characteristic of type 1 neurofibromatosis. The aim of this study is to describe a less frequent complication of the disease which represents a therapeutic challenge due to its topography and frequency of recurrences. This is the case of a 3 year-old girl with type 1 neurofibromatosis with symptoms of multisegmental myelopathy. A cervical-dorsal tumor with medullary compression was shown in medical imaging. Tumor extraction allowed to confirm intradural, extramedullary neurofibroma. Plexiform neurofibromas are characterized by their infiltrative behavior and extensive paraspinal dissemination. Resection of these lesions usually generates morbidity associated with the involvement of extra-spinal critical structures and hinders their complete resection as occurred in the presented clinical case. It is important to perform a correct clinical and eventually imaging follow-up of patients with NF-1 to detect and treat tumors, its most important complication.

Neurofibromatose é a syndrome neurocutânea mais freqüente, uma doença hereditária com envolvimento multissistêmico. Três formas clínicas da doença são distinguidas. A neurofibromatose tipo 1 (NF-1) é a mais freqüente. As lesões cutâneas, presentes na maioria dos casos, podem ser associadas a tumores em qualquer localização. Neurofibromas são característicos de NF-1. O objetivo desta comunicação é descrever uma complicação rara de NF-1 que representa um desafio terapêutico devido à topografia e freqüência de recidivas. Trata-se de uma menina de 3 anos com NF-1 com sintomas de mielopatia multisegmentar de instalação progressiva de dois meses de evolução. A imagem mostrou um tumor cérvico-dorsal com compressão da medula espinhal. A excisão do tumor permitiu confirmar neurofibroma intradural, extramedular. Os neurofibromes plexiformes são caracterizados pelo seu comportamento infiltrativo e sua disseminação paraspinal extensa. A ressecção dessas lesões geralmente gera morbidade associada ao envolvimento de estruturas críticas extra-espinhais e dificulta sua ressecção completa, como ocorreu no caso clínico apresentado. Destaca-se a importância de realizar um acompanhamento clínico e, eventualmente, de imagens de pacientes com NF-1 para detectar e tratar oportunamente os tumores, sua complicação mais importante.

Alterações motoras em crianças e adolescentes pós-cirurgia de tumor intramedular: estudo retrospectivo / Motor changes in children and adolescents after intramedullary tumor surgery: a retrospective study

Introdução: Os tumores intramedulares são raros e correspondem a menos de 10% das neoplasias do sistema nervoso central. O crescimento tumoral pode comprimir feixes nervosos e resultar em perda da função motora e sensorial. A abordagem cirúrgica é o principal pilar de tratamento e visa à máxima ressecção tumoral com preservação da função. A reabilitação destes pacientes é individualizada se analisarmos os déficits funcionais e prognósticos. Objetivo: Analisar a idade e sexo dos pacientes, tipo e localização do tumor, tratamento cirúrgico e tratamento oncológico dos pacientes com tumor intramedular. Descrever as alterações motoras decorrentes desse tratamento. Método: Estudo retrospectivo realizado no Instituto de Oncologia Pediátrica através de dados de prontuários de janeiro de 2013 a dezembro de 2016 de pacientes com tumor intramedular. Dados analisados: idade ao diagnóstico e no momento cirúrgico, gênero, diagnóstico e localização do tumor, tipo de tratamento oncológico, cirurgia realizada, sequela pré e pós-cirurgia, indicação de coletes ortopédicos e tempo de uso. Não houve necessidade de aplicação do Termo de Consentimento Livre e Esclarecido por ser um estudo retrospectivo. Resultados: Foram selecionados doze prontuários de pacientes. A média de idade dos pacientes ao diagnóstico foi de 10 anos e 8 meses e média de 32 dias até a abordagem cirúrgica. Sete pacientes eram do sexo feminino e cinco do sexo masculino. Todos os pacientes foram submetidos a laminectomias, sendo três submetidos também a protocolo quimioterápico e radioterápico, e dois a protocolo quimioterápico. Dois pacientes foram orientados a usar colar cervical e dois orientados a usar colete ortopédico. Todos pacientes apresentaram alteração da marcha e diminuição de força muscular nos grupos pré e pós-cirúrgico. Conclusão: A idade média dos pacientes ao diagnóstico de tumor intramedular foi inferior a 11 anos, com predomínio do sexo feminino. Os diagnósticos oncológicos e as localizações tumorais foram variados. Sete pacientes evoluíram com deambulação independente após atendimento fisioterápico. A indicação de colares e coletes cervicais precisa ser mais estudada e sistematizada.(AU)

Introduction: Intramedullary tumors are rare and account for less than 10% of neoplasms of the central nervous system. Tumor growth may compress nerve bundles and result in loss of motor and sensory function. The surgical approach is the main pillar of treatment and aims at maximum tumor resection with preservation of function. The rehabilitation of these patients is individualized if we analyze the functional and prognostic deficits. Objective: To analyze the age and sex of the patients, type and location of the tumor, surgical treatment and oncological treatment of patients with intramedullary tumor. Describe the motor changes resulting from this treatment. Method: Retrospective study conducted at the Pediatric Oncology Institute through data from medical records from January 2013 to December 2016 of patients with intramedullary tumor. Data analyzed: age at diagnosis and at the time of surgery, gender, diagnosis and location of the tumor, type of cancer treatment, surgery performed, pre and post-surgery sequelae, indication of orthopedic vests and time of use. There was no need to apply the Free and Informed Consent Term because it is a retrospective study. Results: Twelve patient charts were selected. The mean age of the patients at diagnosis was 10 years and 8 months and mean of 32 days until the surgical approach. Seven patients were female and five were male. All patients underwent laminectomies, three of which were submitted to chemotherapy and radiotherapy, and two to chemotherapy protocol. Two patients were instructed to wear cervical collar and two oriented to wear orthopedic vest. All patients presented gait alteration and decreased muscle strength in the pre and post-surgical groups. Conclusion: The mean age of the patients at the diagnosis of intramedullary tumor was less than 11 years, with a predominance of females. Oncological diagnoses and tumor sites were varied. Seven patients evolved with independent walking after physical therapy. The indication of necklaces and cervical vests needs to be further studied and systematized.(AU)

Treatment of Intradural Spinal Arteriovenous Fistula: Technical Case Report

Introduction Although rare, arteriovenous fistula (AVF) is the most common vascular malformation of the spine, and it is mainly located in the thoracic region. The fistula is identified by arteriography, which enables the treatment by embolization or guides the microsurgical therapy. Clinical Presentation We describe the case of a 61-year-old woman with neurogenic claudication evolving to paraparesis. A dorsal intradural AVF was identified by magnetic resonance imaging (MRI) and arteriography, and it was submitted to an embolization, but the procedure was unsuccessful. The patient was then referred for neurosurgery, and the AVF was obliterated using a microsurgical technique guided by multimodal intraoperative monitoring. We identified the AVF in the intraoperative moment and we then cut and coagulated the extradural portion of the nerve root. Discussion Arteriovenous fistulas occur in the dura mater of the nerve roots with the arterialization of the venous plexus. The treatment prevents the progression of the deficits. While performing diagnostic arteriography, attempts of embolization are possible. Conclusion Although the initial attempt of embolization at the moment of the diagnostic arteriography is achievable, it has failure rates of 50%, unlike the surgical treatment, which is definitive in all cases, as reported in this article.

Introdução Apesar de raras, as fístulas arteriovenosas (FAV) são as malformações vasculares mais comuns na coluna, localizadas essencialmente na região torácica. A fístula é identificada por arteriografia, a qual permite o tratamento da embolização ou guia a terapia microcirúrgica. Apresentação Clínica Descrevemos o caso de uma mulher de 61 anos com claudicação neurogênica evoluindo para paraparesia. Uma FAV intradural dorsal foi identificada por meio de ressonância magnética (RM) e arteriografia, e foi submetida a uma embolização, mas o procedimento não obteve sucesso. A paciente foi então encaminhada para tratamento neurocirúrgico, e a FAV foi eliminada por técnica microcirúrgica guiada por monitoração multimodal intraoperatória. Identificamos a FAV durante o período intraoperatório, e em seguida cortamos e coagulamos a porção extradural da raiz do nervo. Discussão As AVFs ocorrem na dura-máter das raízes neurais com a arterialização do plexo venoso. O tratamento evita o progresso de déficits. Durante a arteriografia diagnóstica, tentativas de embolização são possíveis. Conclusão Apesar de a tentativa inicial de embolização durante a arteriografia ser possível, ela tem probabilidades de 50% de sucesso, enquanto o tratamento cirúrgico é definitivo em todos os casos, como relatado neste artigo.

Spinal pleomorphic xanthoastrocytoma: case report and literature review / Xantoastrocitoma pleomórfico espinal: reporte de caso y revisión de la literatura

El Xantoastrocitoma Pleomórfico Espinal (XAPE) es una neoplasia primaria infrecuente del sistema nervioso central, descrita por primer vez en 1979. De estirpe astrocítica y aunque clasificada como grado II por la OMS, pueden existir variantes anaplásicas o malignas. Con localización usualmente temporal, pocos casos espinales se han descrito a la fecha, debido probablemente a un subdiagnóstico o un subregistro. El pronóstico es generalmente bueno y depende en gran medida del tipo histológico y extensión de la resección quirurgica. Describimos el caso de un hombre de 30 años de edad con dorsalgiay paresia del miembro inferior izquierdo. Se encontró en imágenes de resonancia magnética una lesión aparentemente intradural, extraaxial en los segmentos T8-T9 que se llevó a resección. Se confirmó por histopatología la presencia de un XAPE primario. En la presente publicación realizamos una revisión de la literatura disponible.

Spinal Pleomorphic Xanthoastrocytoma (SPXA) is a rare CNS primary neoplasm, first described in 1979. Although of astrocytic lineage and classified as a grade II neoplasm by the WHO, it may be have anaplastic or malignant variants. Usually located in the temporal lobe, few spinal cases have been described to date, probably due to underdiagnosis and underreporting. It usually has a good prognosis, but it depends on its histological type and extent of surgical resection. In this article, we describe the case of a 30-year old male who complained of low back pain and left lower limb paresis. The MRI showed an apparently intradural, extraaxial lesion at T8-T9 segments. The diagnosis of a primary SPXA was confirmed by histopathological studies. In this article, a review of the available literature is presented.

Foix-Alajouanine syndrome mimicking a spinal cord tumor / Síndrome de Foix-Alajouanine simulando um tumor intramedular espinal

Summary Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely reported in the scope of AVF, which poses a diagnostic challenge and raises the suspicion of a spinal cord tumor. In such situations, biopsy can be required and delay proper diagnosis. We report the case of a patient with SNM, who underwent biopsy on the assumption of it being a spinal cord tumor.

Resumo Mielopatia necrotizante subaguda (MNS) ou síndrome de Foix-Alajouanine é uma doença rara que se caracteriza por disfunção neurológica progressiva causada por uma fístula arteriovenosa espinal dural. O diagnóstico radiológico é comumente suspeitado quando aparece captação não específica de contraste e de artefatos de fluxo (flow voids) perimedulares. Raramente, a captação de contraste exibe o aspecto em anel, constituindo um grande desafio diagnóstico. Nesses casos, o principal diagnóstico diferencial é um tumor intramedular, e os pacientes são encaminhados para biópsia da lesão, atrasando o diagnóstico definitivo. Relatamos o caso de uma paciente com MNS, a qual foi submetida à biópsia da lesão em virtude de suspeita de tumor intramedular.

Quiste aracnoidal cervical anterior en edad pediátrica / Anterior cervical arachnoid cyst in pediatric age

Los quistes aracnoidales espinales son lesiones poco comunes en la población pediátrica. La mayor parte de ellos, se ubican en los segmentos dorsales y la posición anterior respecto a la médula es rara en todos los casos. Si bien su patogenia no está aclarada, se han asociado a defectos del tubo neural y traumas previos. Clínicamente, pueden presentarse con síndrome medular que en ocasiones pueden empeorar con cambios posturales. El tratamiento, puede ser conservador o quirúrgico, el que está indicado en presencia de síntomas neurológicos secundarios a compresión medular, siendo el abordaje posterior el más frecuentemente utilizado. El propósito de la cirugía es la resección total o en su defecto, la fenestración del quiste para comunicarlo al espacio subaracnoídeo. Una potencial complicación de la vía posterior, es la herniación medular durante la durotomía, secundaria al efecto compresivo del quiste, la cual podría aumentar la morbilidad neurológica en el período postoperatorio. Se presentan 2 casos consecutivos en edad pediátrica con quistes intradurales espinales anteriores, el primero en la región cervico-dorsal cuya cirugía se vio dificultada por la presencia de herniación medular transdural y un segundo caso con un quiste exclusivamente cervical, en que mediante una punción lateral del quiste guiada por ecografía previo a la durotomía, se logró resecar la lesión sin esta complicación.

Spinal arachnoid cysts are rare lesions in pediatric population. Most of them are located posteriorly in dorsal segments ananterior position is rare. Although its pathogenesis has not been elucidated, they have been associated with neural tube defects and the presence of previous spinal traumas. Clinically, they present with a spinal cord syndrome which can sometimes worsen with postural changes. Treatment may be conservative or surgical, the latter indicated by the presence of neurological symptoms secondary to spinal cord compression, with the posterior approach being the most frequently used. The purpose of surgery is total or partial resection, or fenestration of the cyst to subarachnoid space. A potential intra-surgical complication of posterior approach in anterior cyst is spinal cord herniation during durotomy, secondary to the compressive effect of the cyst, which could increase neurological morbidity in the postoperative period (1 case with mortality is described in the literature). We present 2 consecutive cases in pediatric patients with previous spinal intradural cysts. The first in the cervico-dorsal region whose surgery was hampered by the presence of medullary transdural herniation and second case with an exclusively cervical cyst that through a side puncture cyst guided by ultrasound prior to durotomy, it was possible to resect the lesion without this complication.

Gemistocytic astrocytoma in the spinal cord in a dog: a case report / Astrocitoma gemistocítico medular em cão: relato de caso

This paper reports a case of a rare variant of the cervical spinal cord astrocytoma diagnosed in a dog with progressive neurological signs, initially asymmetrical, not ambulatory tetraparesis, segmental reflexes and normal muscle tone in all four limbs and absence of pain upon palpation of the cervical spine. Myelography revealed attenuation of the ventral and dorsal contrast line in the third region of the fifth cervical vertebra. At necropsy intramedullary cylindrical mass that stretched from the third to the sixth cervical vertebra, which replaced all the gray matter of the spinal cord was observed. In the histological study, there was the replacement of the substance by neoplastic cells mantle arranged loosely. The cells were large and slightly rounded. The eosinophilic cytoplasm was well defined, sometimes forming processes interconnecting cells. The nucleus was eccentric, round, oval or kidney-shaped, and the nucleolus was evident. Thus, the microscopic changes observed in the cervical spinal cord were consistent with gemistocytic astrocytoma.(AU)

Relata-se um caso de uma variante rara de astrocitoma na medula cervical, diagnosticado em cadela com sinais neurológicos progressivos, inicialmente assimétricos, de tetraparesia não ambulatória, com reflexos segmentares e tônus muscular normais nos quatro membros e ausência de dor à palpação da coluna cervical. A mielografia revelou atenuação da linha de contraste ventral e dorsal na região da terceira à quinta vértebra cervical. À necropsia, foi observada massa cilíndrica intramedular que se estendia da terceira à sexta vértebra cervical, a qual substituía toda a substância cinzenta da medula espinhal. No estudo histológico, observou-se substituição da substância por manto de células neoplásicas arranjadas frouxamente. As células eram grandes e levemente arredondadas. O citoplasma eosinofílico, bem delineado, por vezes formava processos interligando as células. O núcleo era excêntrico, redondo, oval ou reniforme, e o nucléolo evidente. Logo, as alterações microscópicas observadas na medula espinhal cervical foram compatíveis com astrocitoma gemistocítico.(AU)