Clinical Characteristics and Surgical Outcomes of 2542 Patients with Spinal Schwannomas: A Systematic Review and Meta-Analysis.

OBJECTIVE: This study was conducted to systematically analyze the data on the clinical features, surgical treatment, and outcomes of spinal schwannomas. METHODS: We conducted a systematic review and meta-analysis under the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A search of bibliographic databases from January 1, 2001, to May 31, 2021, yielded 4489 studies. Twenty-six articles were included in our final qualitative systematic review and quantitative meta-analysis. RESULTS: Analysis of 2542 adult patients' data from 26 included studies showed that 53.5% were male, and the mean age ranged from 35.8 to 57.1 years. The most common tumor location was the cervical spine (34.2%), followed by the thoracic spine (26.2%) and the lumbar spine (18.5%). Symptom severity was the most common indicator for surgical treatment, with the most common symptoms being segmental back pain, sensory/motor deficits, and urinary dysfunction. Among all patients analyzed, 93.8% were treated with gross total resection, which was associated with better prognosis and less chance of recurrence than subtotal resection. The posterior approach was the most common (87.4% of patients). The average operative time was 4.53 hours (95% confidence interval [CI], 3.18-6.48); the average intraoperative blood loss was 451.88 mL (95% CI, 169.60-1203.95). The pooled follow-up duration was 40.6 months (95% CI, 31.04-53.07). The schwannoma recurrence rate was 5.3%. Complications were particularly low and included cerebrospinal fluid leakage, wound infection, and the sensory-motor deficits. Most of the patients experienced complete recovery or significant improvement of preoperative neurological deficits and pain symptoms. CONCLUSIONS: Our analysis suggests that segmental back pain, sensory/motor deficits, and urinary dysfunction are the most common symptoms of spinal schwannomas. Surgical resection is the treatment of choice with overall good reported outcomes and particularly low complication rates. gross total resection offers the best prognosis with the slightest chance of tumor recurrence and minimal risk of complications.

Teratoma quístico intrarraquídeo en un adulto / Cystic Intraspinal Teratoma

RESUMEN El teratoma es una lesión congénita, pero los más pequeños no son descubiertos hasta mucho más tarde en el transcurso de la vida de la persona. Un teratoma maduro es típicamente benigno y se halla más frecuentemente en mujeres y un teratoma inmaduro es maligno y se encuentra con más frecuencia en varones. Presentar un caso no frecuente de un tumor intraespinal en un adulto. Se presenta un caso con un teratoma intrarraquídeo, subdural extramedular a nivel de T7-T8 en un adulto que resulta una rareza por su histología y su localización. Este tumor encapsulado contiene tejidos u órganos que recuerda los derivados normales de las tres capas germinales. Dentro de su cápsula se puede observar uno o más quistes y son generalmente benignos. Este paciente debutó con un síndrome paraparético con trastornos sensitivos y esfinterianos, por lo que fue ingresado de urgencia y se realizaron estudios simples de imagen y resonancia magnética diagnosticándose un tumor intrarraquídeo a nivel dorsal. Los teratomas espinales se comportan como un proceso expansivo; su tratamiento de elección es el quirúrgico. El paciente a los 6 meses de operado logró la marcha con más facilidad con ayuda de un bastón y los trastornos sensitivos y esfinterianos desaparecieron.

ABSTRACT The teratoma is a lesion that is present at birth, congenital, but the smallest are not discovered until much later in the course of the person's life. A mature teratoma is typically benign and is found more often in women, and an immature teratoma is malignant and is found more often in men. To present an unusual case of an intraspinal tumor in an adult. A case is presented with an intraspinal, subdural-extramedullary teratoma at the level of T7-T8 in an adult, which is a rarity due to its histology and location. This encapsulated tumor contains tissues or organs resembling normal derivatives of the three germ layers. Within its capsule one or more cysts can be observed and they are generally benign. This patient debuted with a paraparetic syndrome with sensory and sphincter disorders and was admitted to the emergency department. Simple imaging studies and Magnetic Resonance Imaging were performed, diagnosing an intraspinal tumor at the dorsal level. Spinal teratomas behave as an expansive process; His treatment of choice is surgery. Six months after surgery, the patient was able to walk more easily with the help of a cane, and the sensory and sphincter disorders disappeared.

Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. They are usually located intracranially and rarely in the spinal cord. There is no clear correlation between this tumor and neurofibromatosis type 1 (NF1) with only four cases described. The aim of this article is to describe one more case and add data to the literature regarding this rare association. CASE DESCRIPTION: An 8-year-old boy with NF1 presented progressive asymmetrical paraparesis (Grade 4 medical research council scale on the right leg and Grade 3 on the left leg). The cervicothoracic spinal magnetic resonance imaging demonstrated an intramedullary lesion from C4 to T4 vertebrae. The patient underwent a microsurgical resection. A partial resection was performed due to a drop in the motor evoked potential signal amplitude during dissection. Pathology report revealed a ganglioglioma (World Health Organization Grade I). Postoperatively, the patient evolved with worsening of the paraparesis. A few weeks later, he has improved his preoperative functional neurological state (better strength and gait). Adjuvant radiotherapy was not used. The patient is being followed up at the neurosurgery outpatient clinic. CONCLUSION: This is another case of spinal ganglioglioma associated with NF1. The tumor must be included in the differential diagnosis of patients with NF1 and spinal lesions. Complete microsurgical resection remains the standard treatment for spinal gangliogliomas, however, in this specific case, it was decided to leave a portion of the tumor to prevent neurological damage. The prognosis and treatment of this condition associated with NF1 remains to be determined.

Manejo quirúrgico de ependimomas en médula cervical: A propósito de un caso clínico / Surgical management of ependymomas in the cervical spine: Apropos of a clinical case

Introducción: los ependimomas son tumores cerebrales que surgen de células ependimarias, células de soporte en el cerebro y la médula espinal. Representan entre el 2 y el 3% de todos los tumores cerebrales primarios. Son el cuarto tumor cerebral más común en los niños, donde el 90% de los mismos se localizan en la fosa posterior. En adultos, el 60% de estos tumores se encuentran en la médula espinal pudiendo presentarse a cualquier nivel de ésta; el caso a continuación reporta un ependimoma cervical. Objetivo: reportar un caso de ependimoma medular de ubicación poco frecuente, con resección total, sin recidiva porterior a 2 años de seguimiento.Descripción del caso: se reporta el caso de un paciente adulto, 44 años, masculino, con un síndrome medular cervical completo, progresivo, provocado por un ependimoma cervical. Intervención: se realizó laminectomía de dos espacios [C7 a T1], apertura dural, mielotomía posterior logrando exéresis total de la lesión con durorrafia a sello de agua y posterior cierre por planos sin complicaciones, con seguimiento de 2 años de sobrevida. Conclusiones: la resección total macroscópica de este tipo de tumores es muy importante para poder evitar residiva. El ependimoma cervical puede recidivar, sobre todo cuando existen residuales de la lesión. El manejo oportuno depende de gran manera de que los pacientes acudan tempranamente a valoración especializada; la resección guiada con monitorización neurofisiológica transoperatoria provee mayor oportunidad a los pacientes a no presentar secuelas permanentes y permite asimismo, mejores resultados de la rehabilitación neurológica postoperatoria

Introduction: ependymomas are brain tumors that arise from ependymal cells, supporting cells in the brain and spinal cord. They represent between 2 and 3% of all primary brain tumors. They are the fourth most common brain tumor in children, where 90% of them are located in the posterior fossa. In adults, 60% of these tumors are found in the spinal cord and can occur at any level of the spinal cord; the case below reports a cervical ependymoma. Objective: to report a case of medullary ependymoma of rare location, with total resection, without recurrence after 2 years of follow-up. Case description: a 44-year-old male adult patient with a progressive, complete cervical spinal cord syndrome, caused by a cervical ependymoma, is reported.Intervention: two-space laminectomy [C7 to T1], dural opening and posterior myelotomy were performed, achieving total excision of the lesion with water-seal durorrhaphy and subsequent closure by planes without complications, with a 2-year survival follow-up. Conclusions: macroscopic total resection of this type of tumor is very important to avoid residual. Cervical ependymoma can recur, especially when there is residual lesion. Timely management depends to a great extent on the patients attending early for a specialized evaluation; guided resection with intraoperative neurophysiological monitoring provides a greater opportunity for patients to not present permanent sequelae and also allows better results of postoperative neurological rehabilitation.

Malignant Peripheral Nerve Sheath Tumor Arising Within Lumbar Spinal Plexiform Neurofibroma.

A 41-year-old woman presented with a 16-year history of lumbar pain and right leg paresthesia. Twenty-four years previously, she had been diagnosed with neurofibromatosis type 1 syndrome. Magnetic resonance imaging showed a heterogeneous mass extending through widened lumbar neural foramina to the psoas muscles bilaterally, with a dumbbell-like appearance. Biopsy led to the diagnosis of a malignant peripheral nerve sheath tumor, an uncommon soft tissue sarcoma of neural origin that may occur in the context of neurofibromatosis type 1 syndrome. Given the aggressive behavior of these tumors, prompt and accurate diagnosis is critical to improve patient outcomes.

Spinal thoracic extradural angiolipoma manifesting as acute onset of paraparesis: Case report and review of literature.

BACKGROUND: Angiolipomas are benign tumors most commonly found in the thoracic spine. They are composed of mature adipocytes and abnormal vascular elements that usually present with a slowly progressive course of neurological deterioration. CASE DESCRIPTION: A 35-year-old female, with a prior history of back pain, acutely developed paraparesis. When the thoracic magnetic resonance imaging (MRI) revealed a dorsal epidural mass at the T3-T5 level, she underwent a laminectomy for gross total excision of the lesion that proved to be an angiolipoma. On the second postoperative day, the patient was again able to ambulate. CONCLUSION: The angiolipomas of spine are rare causes of spinal cord compression, and those presenting with acute neurological deficits should be immediately treated.

Condrosarcoma de la primera vértebra cervical. Reporte de caso / Condrosarcoma of the first cervical vertebra. Case report

Fundamento: Los tumores primarios de la columna vertebral son raros, con una incidencia estimada de 2.8-8.5 por cada 100 000 individuos anualmente. El condrosarcoma es la segunda neoplasia ósea más frecuente de los tumores óseos primarios malignos, presentándose principalmente en el esqueleto axial: 45 %; siendo muy raros en los huesos craneofaciales y la columna. Objetivo: Describir la presentación clínica de un caso con un condrosarcoma en la primera vértebra cervical y la conducta quirúrgica tomada una vez diagnosticado Presentación de caso: Paciente masculino de 86 años de edad con antecedentes de hipertensión arterial controlada farmacológicamente, que comienza con cervicalgia de difícil tratamiento, tortícolis y disfagia a los sólidos. Se diagnostica mediante TAC de unión cráneo-espinal lesión tumoral en C1. Se le realiza exéresis parcial del tumor con instrumentación occipito-cervical, el paciente evoluciona satisfactoriamente. Conclusiones: El condrosarcoma es un tumor de crecimiento lento, de características variables. La localización espinal es muy rara. Su tratamiento incluye la resección de la lesión con instrumentación si se desestabiliza la columna vertebral, además de quimioterapia y radioterapia de ser necesario.

Background: The primary tumors of the spine are strange, with a dear incidence of 2.8-8.5 of each 100 000 individuals annually. The condrosarcoma is the second more frequent bony neoplasia of the wicked primary bony tumors, being presented mainly in the axial skeleton: 45%; being very strange in the bones craneofacials and the column. Objective: To describe the clinical presentation of a case with a condrosarcoma in the first cervical vertebra and the surgical behavior taken once diagnosed. Case presentation: 86 years old masculine patient with antecedents of pharmacologically controlled hypertension that it begins with cervicalgia of difficult treatment, torticollis and disfagia to the solids. It is diagnosed by means of TAC of union skull-spinal lesion tumoral in C1.a partial exeresis of the tumor with occipito-cervical instrumentation is carried out, the patient evolves satisfactorily. Conclusions: The condrosarcoma is a tumor of slow growth, of variable characteristics. The spinal localization is very strange. Their treatment includes the resection of the lesion with instrumentation if the spine is destabilized, besides chemotherapy and radiotherapy of being necessary.

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.(AU)

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female ¹ mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.(AU)

Lesiones intramedulares que simulan tumor / Spinal cord lesions that mimic neoplasms

Objetivo. Analizar las características clínicas y neurorradiológicas de las distintas lesiones intramedulares con la finalidad de poder diferenciar las lesiones no-tumorales de las tumorales y reservar la exploración quirúrgica sólo para aquellos pacientes sin diagnósticoy con sintomatología rápidamente progresiva. Material y métodos. Se revisaron las historias clínicas de 44 pacientes operados consecutivamente con diagnóstico presuntivo de tumor intramedular entre enero de 1988 y diciembre de 2007. No se incluyeron en el análisis los tumores bulbomedulares ni los del cono y filum ni tampoco los lipomas, teratomas y quistes dermoides.Resultados. Se identificaron 4 pacientes (9.1%), 3 masculinos y 1 femenino, con edad media de 9 años, que presentaban deterioro neurológico progresivo e IRM espinal compatible con tumor intramedular. El resultado histopatológico confirmó en los cuatro casos lesiones no tumorales: infiltrado inflamatorio inespecífico, edema, micosis y gliosis respectivamente.Conclusiones. El minucioso análisis clínico, de laboratorio y el estudio de las imágenes en IRM puede ayudar a la correcta distinción entre tumores y lesiones no-tumorales evitando la exploración quirúrgica que sólo está justificada en los casos muy dudososacompañados de rápido deterioro neurológico funcional del paciente.

Objective. To analyze the clinic and neuro-imaging characteristicsof the intra spinal lesions in order to differentiate the non-neoplasticlesions from neoplastic lesions and to spare surgical exploration only for non-diagnosed patients with rapid deterioration. Materials and method. Fourty-four medical charts of patientsoperated on spinal cord lesions between January 1988 and December 2007 have been analyzed. Results. In four patients (3 male, 1 female – mean age: 9 years)who were operated with diagnosis of intra-spinal tumor, their lesions turned out to be non-neoplastic: non-specific inflammation, edema, mycosis and gliosis, respectively. Conclusions. The thorough clinical and laboratory investigation together with the study of the imaging can help distinguishbetween non-neoplastic lesions from neoplastic lesions in order to avoid surgical exploration which is only suitable in very doubtful cases with rapid deterioration.

Primary filum terminale ependymoma: a series of 16 cases / Ependimoma primário de filum terminale: análise de uma série de 16 casos

Filum terminale ependymomas are slow growing tumors of the cauda equina with a high incidence in young adults. Although a complete microsurgical resection can lead to a cure, recurrence is not uncommon. Sixteen cases of filum terminale ependymomas treated at the Instituto de Neurologia de Curitiba were analyzed. Eleven patients were females and 5 males, their age ranging from 7 to 84 years. Symptoms and signs included lumbar pain (31.25 percent), radicular pain (56.25 percent) and neurological deficits (12.5 percent). In three cases, patients had previously undergone surgery in other hospitals. All were tested through MRI and were operated on. Two underwent a laminoplasty and 14 a laminectomy. The last 8 patients of this series had neuro-physiological monitoring during surgery. In all patients a total microsurgical resection was achieved. Histologically, 2 cases were cellular ependymomas and 14 cases myxopapillary ependymomas. There was no recurrence during a 2 to 84 month follow-up period.

Os ependimomas do filum teminale são tumores da cauda eqüina de crescimento lento com maior incidência em adultos jovens. A ressecção microcirúrgica total possibilita a cura da doença, recidivas, entretanto, apresentam sérias dificuldades no tratamento. Com o objetivo de estudar os aspectos clínicos, anatomopatológicos e do tratamento, analisaram-se 16 casos de ependimomas do filum teminale tratados no Instituto de Neurologia de Curitiba, 11 do sexo feminino e 5 do sexo masculino, com idade entre 7 e 84 anos, que apresentavam dor lombar (31,25 por cento), radiculopatia (56,25 por cento) e déficits neurológicos (12,5 por cento). Em 3 casos, os pacientes tinham sido operados em outro serviço anteriormente. Em todos os casos o diagnóstico foi confirmado pela ressonância magnética. Em 2 pacientes realizou-se laminoplastia e em 14 laminectomia. Nos últimos 8 pacientes empregou-se monitorização neurofisiológica. Em todos os casos a ressecção microcirúrgica foi total. Do ponto de vista histológico, demonstraram-se 2 casos de ependimoma celular e 14 casos mixopapilares. Não houve recidiva do tumor em um seguimento entre 2 e 84 meses.

Manejo quirúrgico en un paciente con neurofibromatosis tipo 1 / Surgical management in a patient with neurofibromatosis type 1

El presente trabajo tiene por objetivo dar a conocer el manejo de una paciente con una enfermedad genética del grupo de las facomatosis, con predominio de lesiones espinales. Se trata de una mujer de la tercera edad, que durante su desarrollo infantil y puberal le aparecieron lesiones cutáneas en forma de manchas y tumores pediculados distribuidos en todo el cuerpo; posteriormente se instauró una paraparesia espástica. A los 3 años de encamada se ingresó en Instituto Superior de Medicina Militar "Dr. Luis Díaz Soto" y se estudió. La resonancia magnética nuclear realizada diagnosticó 2 lesiones hiperintensas intrarraquídeas, una de localización cervical que le ocasionaba compresión medular, por lo cual se le realizó una laminectomía con exéresis de la lesión y la consiguiente recuperación neurológica. La paciente volvió a caminar, recuperó sensibilidad propioceptiva y se encuentra pendiente de la intervención de la otra lesión en región craneoespinal. El informe patológico concluyó neurofibroma. Con este caso presentado se demuestra que la resección de los neurofibromas espinales en los pacientes con neurofibromatosis tipo I, permite una recuperación de las funciones neurológicas afectadas por el compromiso neural.

This paper is aimed at making known the management of a female patient with a genetic disease of the phacomatosys group, with predominance of spinal injuries. This elderly woman during her childhood and puberty presented skin lesions in the form of spots and pediculated tumors all over her body. Later on, she developed a spastic paraparesia. Alter being bedridden for 3 years, she was admitted at "Dr. Luis Díaz Soto" Higher Military Medicine Institute to be studied. Two intrarachidian hypertensive lesions were diagnosed by magnetic resonance imaging. One of them was a cervical lesion that caused her a medullary compression that was treated by laminectomy with exeresis of the lesion and the subsequent neurological recovery. The patient walked again and recovered her proprioceptive sensitivity and she has to be operated on of the other lesion in the craniospinal region. The pathological report confirmed the existence of a neurofibroma. On presenting this case, it was proved that the resection of the spinal neurofibroma in patients with type I neurofibromatosis allows a recovery of the neurological functions affected by the neural compromise.

"La obstrucción estaba abajo": tumores de la médula espinal e hipertensión intracraneal

Aumento de la presión intracraneal, hidrocéfalo y papiledema son observados ocasionalmente en pacientes portadores de tumores medulares en la región cervical o en la unión craneocervical, y entre otros, la obstrucción mecánica de la circulación del líquido cefalorraquídeo es asumida como responsable para tales síntomas y signos. No obstante, la hipertensión intracraneal es un raro fenómeno en tumores espinales de localización tóracolumbar. Los autores describen dos pacientes en quienes un tumor en tal localización (schwannoma benigno y paraganglioma) se asoció a síntomas de hipertensión intracraneal, hidrocefalia y papiledema. Se piensa que este tipo de lesiones puede interferir activamente en la dinámica del flujo del líquido cefalorraquídeo en razón de que la ocupación de espacio impide la expansión del saco dural y espacio subaracnoideo espinal que se piensa juegan algún papel en la compensación de los cambios de volumen y la presión intracraneal

Increased intracranial pressure, hydrocephalus and papilledema are occasionally observed in patients harboring spinal tumors in the cervical region or at the craniocervical junction, and among others, the mechanical obstruction to the cerebrospinal fluid circulation is assumed to be responsible for such symptoms and signs. However, increased intracranial pressure is very rare in spinal tumors located in the thoraco-lumbar region. Two patients are described by the authors in whom a benign thoracolumbar tumor (benign schwannoma and paraganglioma) was associated with symptoms of increased intracranial pressure, hydrocephalus and papiledema. It has been suggested that this kind of lesions could actively interfere with cerebrospinal fluid dynamics by their mass effect, especially by preventing the expansion of the dural sac and spinal subarachnoid space which may play some roce in compensating for volume changes and intracranial pressure