Ganglioglioma espinal intramedular: caso clínico y revisión comparativa de la literatura / Intramedullary spinal cord ganglioglioma: Case report and comparative literature review

Introducción Los gangliogliomas espinales intramedulares (GGEI) representan el 35-40% de todos los tumores intramedulares en niños. Estos tumores presentan un algoritmo terapéutico y pronóstico diferente con respecto a otros tumores medulares, tales como los astrocitomas o los ependimomas espinales. El objetivo del estudio es revisar la literatura para esclarecer una vía diagnóstica y terapéutica de dicho tumor en base a un caso clínico de GGEI diagnosticado en nuestro centro. Método Se ha realizado una revisión literaria exhaustiva de los GGEI publicados desde 1911 hasta 2018 a través de la plataforma PubMed-NCBI, adjuntando de cada paciente, las características epidemiológicas, la localización, la clínica, el diagnóstico radiológico y el tratamiento. Resultados Se encontraron un total de 167 casos de GGEI, incluyendo nuestro caso. En nuestra muestra, el 52% de los pacientes pertenece al sexo femenino, siendo la década de edad más afectada la que va de los 0 a los 9 años (35% de los pacientes). El déficit motor se establece como síntoma principal en pacientes adultos en mayor proporción que en pacientes pediátricos. Radiológicamente, este tumor se presenta con mayor hiperintensidad y captación de contraste que los astrocitomas y los ependimomas, así como un mayor porcentaje de quistes intratumorales. La mutación BRAFV600E es menos frecuente en los gangliogliomas espinales que en los supratentoriales. La cirugía con resección total es el tratamiento de elección. Solamente el 19% de los pacientes de la muestra recibieron radioterapia y solo el 9% quimioterapia como única vía de tratamiento. Conclusiones Los GGEI son frecuentes en la población pediátrica y requieren de un alto nivel de sospecha para su correcto diagnóstico y tratamiento, ya que tienen 3 veces más riesgo de recurrencia que sus homólogos supratentoriales (AU)

Introduction Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center. Method An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended. Results A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAFV600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. Conclusions ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas (AU)

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.

Intramedullary spinal cord ganglioglioma: Case report and comparative literature review. / Ganglioglioma espinal intramedular: caso clínico y revisión comparativa de la literatura.

INTRODUCTION: Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center. METHOD: An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended. RESULTS: A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAFV600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. CONCLUSIONS: ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas.

Paravertebral lipomatous ganglioneuroma with intraspinal extension. Case report and literature review. / Ganglioneuroma lipomatoso paravertebral con extensión intraespinal. Descripción de un caso y revisión de la literatura.

Lipomatous ganglioneuromas are a rare variant of ganglioneuromas characterized by a mature adipocytic component admixed with a conventional ganglioneuroma component. We present the case of a 34 year old patient with a paravertebral right lesion L1-L4 with intraspinal extension and secondary neurological deficit, who underwent surgery in our hospital. The pathological anatomy showed a yellowish encapsulated neoplasm, which under microscopic evaluation showed areas of ganglioneuroma admixed with areas of mature fat. In the follow up, the patient was asymptomatic, had recovered paresis in the right lower extremity and in the last image control did not present data of lesion recurrence. Fewer than 10 cases of lipomatous ganglioneuromas have been reported in the literature, being this the first paravertebral case wih intraspinal extension and with neurological deficit, hence the interest of this work.

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

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

Quiste neuroentérico intradural: Reporte de un caso / Intradural neurenteric cysts. Case report

El quiste neuroentérico espinal es una lesión de rara presentación, sólo existen reportes de casos aislados. Representa del 0,3% al 1,3% de los tumores espinales según las distintas citas bibliográficas. Suele afectar el canal espinal, especialmente a nivel cervical bajo y torácico superior. Aproximadamente el 90% de estos quistes se localiza en el espacio intradural/ extramedular, mientras que el 10% restante se divide entre la localización intradural/intramedular o extradural. El tratamiento de los quistes neuroentéricos intraespinales es quirúrgico, mediante la resección lo más radical posible. Los resultados son favorables y las tasas de recurrencia después de la resección parcial son bajas. Nivel de Evidencia: IV

Spinal neurenteric cyst is a rare lesion, only anecdotal case reports have been published. The neurenteric cyst accounts for 0.3 to 1.3% of spinal axis tumors. It affects the inferior cervical and superior thoracic spine level. Approximately 90% of neurenteric cysts are located in the intradural/extramedullary compartment while the remaining10% are divided between an intradural/intramedullary or extradural location. Surgical intervention with radical resection is the best treatment. The outcome is favorable and the recurrence rates after partial resection are low. Level of Evidence: IV

[Primary intramedullary melanoma: Case report and literature review]. / Melanoma primario intramedular: reporte de caso y revisión de la literatura.

A dark pigmented intramedullary mass is very rarely encountered in daily practice, and poses a diagnostic challenge. Several entities have to be considered, including melanin-containing tumours (melanotic ependymoma and melanotic schwannoma) and melanocyte-containing tumours (melanocytoma, primary melanoma and melanoma metastases). The case is presented of a 47 year-old male with a pigmented intramedullary tumour located at T7-T8 level. Magnetic resonance images (MRI) revealed a tumour with hyperintensity on T1 and hypointensity on T2. The tumour was resected partially and treated with adjuvant radiotherapy. The diagnosis of primary intramedullary melanoma (PIM) was established based on histology and the absence of other lesions outside of the CNS. A literature review is presented on the other 26 PIM cases reported. PIM are extremely rare tumours, but are the most frequent cause of pigmented intramedullary tumour. Complete surgical resection is the treatment of choice whenever possible, followed by radiotherapy.

[Lumbar spinal angiolipoma]. / Angiolipoma epidural lumbar.

Spinal angiolipomas are fairly infrequent benign tumours that are usually located in the epidural space of the thoracic column and represent 0.14% to 1.3% of all spinal tumours. Lumbar angiolipomas are extremely rare, representing only 9.6% of all spinal extradural angiolipomas. We report the case of a woman who complained of a lumbar pain of several months duration with no neurological focality and that had intensified in the last three days without her having had any injury or made a physical effort. The MR revealed an extradural mass L1-L2, on the posterior face of the medulla, decreasing the anteroposterior diameter of the canal. The patient symptoms improved after surgery. Total extirpation of the lesion is possible in most cases, and the prognosis is excellent even if the lesion is infiltrative. For this reason, excessively aggressive surgery is not necessary to obtain complete resection.

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

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 porciento; 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(AU)

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: 45precent ; 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(AU)

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.

[Spinal osteochondroma: diagnostic imaging and treatment. Case reports]. / Osteocondroma espinal: diagnóstico por imagen y tratamiento. Reporte de casos.

BACKGROUND: Osteochondromas are benign bony tumours, with only 1 to 4% being located in the spine. It occurs more frequently in the cervical spine, with C2 being the vertebra most affected. The neurological presentation is slow due to the growth characteristics of the tumour. Computed axial tomography is the reference method for diagnosis. Surgical management is indicated for patients with neurological impairment or pain. CLINICAL CASE: The first case presents a 21-year-old male with osteochondroma located in the spinous processes of L2, L3 and L4. The second case is a 20-year-old female with multiple osteochondromatosis with tumours at the right lateral mass of C1, with extension to C2 and tumours on the spinous processes of C5 and C7. Both patients presented with painful symptoms, which were resolved after surgical resection of the tumours. CONCLUSIONS: The rarity of these conditions, relevance of a clinical-radiographic diagnosis, and considerations required for surgical treatment are discussed here.

Metástase espinhal intradural e extramedular: relato de caso / Intradural and Extramedular Spinal Metastases: Case Report

Metástases espinhais extramedulares e intradurais são peculiares e correspondem a pequena porcentagem dos casos de tumores da coluna vertebral. Os pacientes normalmente apresentam dor e sintomas neurológicos na primeira apresentação. A imagem por ressonância magnética (RM) é crucial para confirmar a suspeita do diagnóstico. Em geral, a manipulação requer tratamento paliativo representado por radioterapia e/ou cirurgia, para evitar futuros déficits neurológicos. O objetivo do autor é apresentar um caso incomum de metástase espinhal de câncer de mama em uma paciente de 41 anos de idade, com doença sistêmica controlada.

Intradural extramedular spinal metastases are peculiar. They correspond to small percentage of spinal tumors cases. Patients ordinarily present pain and neurological symptoms at the first presentation. Themagnetic resonance image (MRI) scan is crucial to confirm a diagnosis suspicion. On the whole, its handling requires a palliative management represented by radiotherapy and/or surgery to obviate future neurological deficits. The author's aim is present an unusual spinal metastasis of the breast cancer in a 41-year-old female patient with controlled systemic disease.

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.

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.