H3K27M-mutant glioma in thoracic spinal cord and conus medullaris with pilocytic astrocytoma morphology: case report and review of the literature.

BACKGROUND: The H3K27M-mutant spinal cord gliomas are very aggressive with a dismal prognosis, very few cases have been reported in the thoracic spinal cord and conus medullaris, and it is extremely rare with morphological features of pilocytic astrocytoma. CASE PRESENTATION: A 20-year-old man presented with thoracolumbar pain, progressive paraparesis, and urinary incontinence. Magnetic resonance imaging revealed an intramedullary solid-cystic lesion from D9 to conus medullaris. Subtotal resection was performed, restricted by the indistinct margins and the decline of the motor evoked potential during the surgery. Pathologic findings revealed a pilocytic astrocytoma with anaplastic features. However, a further assessment determined a diffuse midline glioma H3K27M-mutant, and adjuvant chemoradiotherapy was administered. After seven months of progression-free survival, the paraparesis worsened; at twelve months of follow-up, the patient developed paraplegia, and at 24 months the patient remains alive without any neurologic functions distal to the tumor and he is still under adjuvant treatment. CONCLUSIONS: The H3K27M-mutant spinal cord glioma is a very infrequent tumor with a wide variety of histological presentations even as indolent as pilocytic astrocytoma, which should be considered in spinal cord tumors, especially if there are clinical, histological, or radiological data that suggest aggressiveness. On the other hand, the fast progression led to the loss of complete neurological function distal to the tumor, in spinal tumors could explain a not so poor prognosis as it is in functionally and vital structures.

Tumor fibroso solitario extra-intramedular de localización dorsal / Solitary fibrous tumor: A clinical case

Introducción: El tumor fibroso solitario (TFS) es un tumor poco frecuente de origen mesenquimal, que se localiza principalmente en pleura. De extraordinaria infrecuencia es su localización a nivel intraespinal, siendo la región torácica la más frecuente. Presentación del caso: Presentamos el caso de una paciente de 48 años con hipoestesia ascendente progresiva en miembros inferiores y mielopatía de un mes de evolución, que se diagnosticó de un tumor intraespinal en segmento D3-D4. Fue intervenida quirúrgicamente mediante abordaje dorsal posterior y laminoplastia D3-D4, hallándose un tumor intradural con componente intramedular, de 18×12mm, aproximadamente. La resección fue completa y la anatomía patológica resultó el diagnóstico de tumor fibroso solitario. La paciente, tras 7 meses de seguimiento se encuentra asintomática. Discusión: La resección completa tumoral junto con las características histopatológicas son los principales factores pronósticos, teniendo la cirugía un papel protagonista en este tipo de neoplasia. Conclusión: Son muy pocos los casos publicados en la literatura de tumor fibroso solitario con localización intraespinal. Con este artículo aportamos un nuevo caso a la misma

Introduction: Solitary fibrous tumor (TFS) is a rare tumor of mesenchymal origin, located mainly in the pleura. It is extraordinarily infrequent find it at the intraespinal level, being the thoracic region the most frequent. Case presentation: We present the case of a 48-year-old patient with progressive ascending lower limb and myelopathy of one month of evolution, with intraspinal location at the D3-D4 level. It was surgically operated by posterior dorsal approach and D3-D4 laminoplasty, with an intradural tumor with an intramedullary component of approximately 18×12mm. The resection was complete and the pathological anatomy gave the diagnosis of solitary fibrous tumor. The patient is currently asymptomatic. Discussion: Complete tumor resection and histopathological features are the main prognostic factors. Surgery have a main role in this type of neoplasia. Conclusion: There are few case published of solitary fibrous tumor with intraspinal localization. We apport another case to the literature

Solitary fibrous tumor: A clinical case. / Tumor fibroso solitario extra-intramedular de localización dorsal.

INTRODUCTION: Solitary fibrous tumor (TFS) is a rare tumor of mesenchymal origin, located mainly in the pleura. It is extraordinarily infrequent find it at the intraespinal level, being the thoracic region the most frequent. CASE PRESENTATION: We present the case of a 48-year-old patient with progressive ascending lower limb and myelopathy of one month of evolution, with intraspinal location at the D3-D4 level. It was surgically operated by posterior dorsal approach and D3-D4 laminoplasty, with an intradural tumor with an intramedullary component of approximately 18×12mm. The resection was complete and the pathological anatomy gave the diagnosis of solitary fibrous tumor. The patient is currently asymptomatic. DISCUSSION: Complete tumor resection and histopathological features are the main prognostic factors. Surgery have a main role in this type of neoplasia. CONCLUSION: There are few case published of solitary fibrous tumor with intraspinal localization. We apport another case to the literature.

Quiste intraneural del nervio supraescapular: causa atípica de síndrome de atrapamiento de nervio periférico. Presentación de un caso y revisión de la literatura / Intraneural cyst of the supraescapular nerve: Atypical cause of peripheral nerve entrapment syndrome. Case report and literature review

Objetivo: Los quistes intraneurales son lesiones benignas de contenido mucinoso que se ubican dentro del epineuro de algunos nervios periféricos, de etiopatogenia controvertida. La mayoría se localizan a nivel de los miembros inferiores. Cuando aparece en los miembros superiores, el nervio más frecuentemente afectado es el cubital. De extraordinaria infrecuencia es el síndrome de atrapamiento del nervio supraescapular debido a la formación de un quiste intraneural del mismo. Con este artículo, aportamos un nuevo caso y realizamos una revisión de la literatura de los quistes intraneurales localizados en el nervio supraescapular. Métodos: Presentamos el caso de una paciente de 49 años con dolor en región lateral del hombro de varios meses de evolución. Se realizó RM plexo braquial donde se objetivó una tumoración de aproximadamente 2×1,5cm, de aspecto quístico, en relación al tronco superior del plexo braquial derecho. Resultados: Fue intervenida bajo control neurofisiológico, mediante un abordaje supra-infraclavicular. La tumoración quística afectaba a la extensión del nervio supraescapular. Tras localizar una zona en la superficie libre de fascículo nervioso se procedió a la disección, vaciamiento del quiste con contenido gelatinoso xantocrómico y resección parcial de la capsula. El examen anatomopatológico confirmó el diagnóstico de quiste intraneural (ganglión). Conclusión: El nervio supraescapular es un nervio mixto que procede del tronco superior. Proporciona las ramas motoras al músculo supraespinoso e infraespinoso, y puede derivar, resultado de la compresión, en una atrofia de los mismos. Es importante conocer esta entidad que, aún de extraordinaria infrecuencia, es uno de los diagnósticos diferenciales que deben ser tenidos en cuenta al estudiar un paciente con dolor irradiado al hombro, y su tratamiento correcto redunda muchas veces en la remisión completa de la sintomatología

Objective: Intraneural cysts are benign lesions located within the epineurium of some peripheral nerves and their aetiopathogenesis is controversial. Most are located at the level of the lower limbs. In the upper limbs, the most frequently affected nerve is the ulnar nerve. Suprascapular nerve entrapment syndrome due to the formation of an intraneural cyst is rare. In this article, we show a new case and perform a literature review of intraneural cysts located in the suprascapular nerve. Methods: We present a 49-year-old woman with pain in the lateral shoulder region of several months’ evolution. A brachial plexus MR showed a tumour of approximately 2×1.5cm, with a cystic appearance, in relation to the upper trunk of the right brachial plexus. Results: We used a supra-infraclavicular approach. The cystic tumour affected the suprascapular nerve. After locating a zone on the surface without nervous fascicles, we performed a partial resection of the capsule and emptying of the cyst, with a xanthochromic gelatinous content. The anatomopathological examination confirmed the diagnosis of intraneural cyst. Conclusion: The suprascapular nerve is a mixed nerve, coming from the upper trunk. It provides the motor branches to the supraspinatus and infraspinatus muscle. Compression of the suprascapular nerve leads to atrophy of these muscles. This entity is one of the differential diagnoses in a patient with pain irradiating to the shoulder, and its correct treatment often results in complete remission of symptoms

Intraneural cyst of the supraescapular nerve: Atypical cause of peripheral nerve entrapment syndrome. Case report and literature review. / Quiste intraneural del nervio supraescapular: causa atípica de síndrome de atrapamiento de nervio periférico. Presentación de un caso y revisión de la literatura.

OBJECTIVE: Intraneural cysts are benign lesions located within the epineurium of some peripheral nerves and their aetiopathogenesis is controversial. Most are located at the level of the lower limbs. In the upper limbs, the most frequently affected nerve is the ulnar nerve. Suprascapular nerve entrapment syndrome due to the formation of an intraneural cyst is rare. In this article, we show a new case and perform a literature review of intraneural cysts located in the suprascapular nerve. METHODS: We present a 49-year-old woman with pain in the lateral shoulder region of several months' evolution. A brachial plexus MR showed a tumour of approximately 2×1.5cm, with a cystic appearance, in relation to the upper trunk of the right brachial plexus. RESULTS: We used a supra-infraclavicular approach. The cystic tumour affected the suprascapular nerve. After locating a zone on the surface without nervous fascicles, we performed a partial resection of the capsule and emptying of the cyst, with a xanthochromic gelatinous content. The anatomopathological examination confirmed the diagnosis of intraneural cyst. CONCLUSION: The suprascapular nerve is a mixed nerve, coming from the upper trunk. It provides the motor branches to the supraspinatus and infraspinatus muscle. Compression of the suprascapular nerve leads to atrophy of these muscles. This entity is one of the differential diagnoses in a patient with pain irradiating to the shoulder, and its correct treatment often results in complete remission of symptoms.

Quistes articulares espinales: evolución clínica y resultados según intervención quirúrgica. Revisión de 18 casos / Spinal sinovial cysts: Surgical treatment and clinical outcomes in a series of 18 cases

Objetivo: Se realiza un análisis retrospectivo a partir de una serie de 18 casos con quistes articulares sintomáticos, tratados quirúrgicamente mediante distintos abordajes para su extirpación desde mayo del 2009 hasta noviembre del 2013. Se muestran los resultados obtenidos con cada uno de los abordajes a medio y largo plazo. Material y métodos: Se incluye a 18 pacientes, 8 varones y 10 mujeres, de entre 50 y los 77 años. Se recogen de cada uno de ellos los síntomas, antecedentes, estudios por imagen, histopatología, intervención quirúrgica, seguimiento, complicaciones y resultados. Resultados: Diecisiete casos fueron diagnosticados de quiste articular sinovial y un tuvo como resultado ganglión. El síntoma más común es la lumbalgia con radiculopatía (94%). La pérdida de fuerza se registró en 4 casos (22%) y un caso (1%) se acompañó de alteración de esfínteres. El nivel más frecuente fue L4-L5 (67%). Recogimos un caso en locación atípica D12-L1. Se practicó una descompresión unilateral del canal en 14 casos mediante hemilaminectomía, colocándose en 9 de ellos dispositivo interespinoso; en 3 casos se realizó laminectomía y artrodesis, y en uno solo se realizó un abordaje unilateral con descompresión bilateral del canal. Todos ellos tuvieron un seguimiento mínimo de 6 meses y máximo de 2 años. Conclusiones: Los quistes articulares son una causa de radiculopatía/claudicación neurógena. La localización más frecuente es el nivel L4-L5 y la forma más frecuente de presentación la lumbalgia con radiculopatía. La resonancia magnética lumbar es la prueba de elección para su diagnóstico. Los quistes articulares refractarios a tratamiento conservador serían subsidiarios de cirugía. En nuestra serie, la extirpación quirúrgica del quiste muestra un buen resultado clínico a medio-largo plazo, si bien la heterogeneidad del grupo y el número de casos no permiten escoger un abordaje óptimo para su resolución. El mejor abordaje quirúrgico para el tratamiento de los quistes articulares sigue siendo controvertido

Object: A series of 18 patients with symptomatic synovial cysts was analysed from May 2009 to November 2013. Different approaches were performed for their removal. Material and methods: The study included 18 patients, 8 men and 10 women, aged between 50 and 77 years. An analysis was made of the variables including age, gender, symptoms, imaging studies, histopathology, surgery, follow-up, complications, and clinical outcome. Results: An articular synovial cyst was diagnosed in 17 cases, and a ganglion in one cases. The most common symptom was back pain with radiculopathy (94%). Motor deficits occurred in 4 cases (22%), and 1 case (5%) presented with urinary incontinence. The most common level was L4- L5 (67%), with one atypical case observed in the D12 -L1 location. Hemi-laminectomy was performed in 14 cases, with 9 of them having an interspinous spacer (ISP) device inserted. A laminectomy with a fusion procedure was performed in 3 patients and 1 patient had a bilateral decompression using a unilateral approach. The patients were followed-up for between 6 months to 2 years. Conclusions: Synovial cysts are a cause of radiculopathy/neurogenic claudication. Spinal cysts are commonly found at the L4-L5 level. MRI is the tool of choice for diagnosis. The most common symptom was back pain with radiculopathy. Synovial cysts resistant to conservative therapy should be treated surgically. In our series, surgical resection of symptomatic juxtafacet cysts showed a good clinical outcome, but the optimal approach for patients with juxtafacet cysts remains unclear

[Spinal sinovial cysts: Surgical treatment and clinical outcomes in a series of 18 cases]. / Quistes articulares espinales: evolución clínica y resultados según intervención quirúrgica. Revisión de 18 casos.

OBJECT: A series of 18 patients with symptomatic synovial cysts was analysed from May 2009 to November 2013. Different approaches were performed for their removal. MATERIAL AND METHODS: The study included 18 patients, 8 men and 10 women, aged between 50 and 77 years. An analysis was made of the variables including age, gender, symptoms, imaging studies, histopathology, surgery, follow-up, complications, and clinical outcome. RESULTS: An articular synovial cyst was diagnosed in 17 cases, and a ganglion in one cases. The most common symptom was back pain with radiculopathy (94%). Motor deficits occurred in 4 cases (22%), and 1 case (5%) presented with urinary incontinence. The most common level was L4- L5 (67%), with one atypical case observed in the D12 -L1 location. Hemi-laminectomy was performed in 14 cases, with 9 of them having an interspinous spacer (ISP) device inserted. A laminectomy with a fusion procedure was performed in 3 patients and 1 patient had a bilateral decompression using a unilateral approach. The patients were followed-up for between 6 months to 2 years. CONCLUSIONS: Synovial cysts are a cause of radiculopathy/neurogenic claudication. Spinal cysts are commonly found at the L4-L5 level. MRI is the tool of choice for diagnosis. The most common symptom was back pain with radiculopathy. Synovial cysts resistant to conservative therapy should be treated surgically. In our series, surgical resection of symptomatic juxtafacet cysts showed a good clinical outcome, but the optimal approach for patients with juxtafacet cysts remains unclear.

Hemangioblastoma radicular extraforaminal de la raíz L4 derecha / Hemangioblastoma of the right L4 nerve root with radicular extraforaminal involvement

Los hemangioblastomas son tumores benignos, hipervasculares, cuya localización más frecuente es a nivel de la fosa posterior o la médula espinal. Los hemangioblastomas radiculares representan menos del 2% del total. Presentamos el caso de un paciente con un cuadro de dolor radicular en el territorio de la raíz L4 derecha a consecuencia de un hemangioblastoma radicular dependiente de dicha raíz. El diagnóstico primario de esta patología suele ser erróneo, sospechándose esta entidad únicamente durante el acto quirúrgico. Dada la gran vascularización que presentan, es aconsejable intentar una embolización prequirúrgica para disminuir así el sangrado intraoperatorio y evitar lesiones en raíces elocuentes. En nuestro caso, tras la devascularización del tumor y bajo control neurofisiológico, se consiguió una extirpación completa de la lesión sin evidenciarse déficit neurológico posterior

Hemangioblastomas are benign hypervascular tumours, which are frequently located at the posterior fossa or the spinal cord. Nerve root hemangioblastomas account for less than 2%of total cases. Sometimes the initial diagnosis can be inaccurate and the final diagnosis is not reached until the surgical procedure is performed. Given the high vascularisation of this particular kind of tumour, preoperative embolisation of the tumour is recommended to reduce surgical bleeding and minimise the risk of injuries to motor nerve roots. In this particular case, the patient presented with radicular pain in the right L4 nerve root territory originated by a radicular extraforaminal hemangioblastoma. After tumour devascularization and under neurophysiological control, total excision of lesion was achieved without posterior neurological deficit

[Hemangioblastoma of the right L4 nerve root with radicular extraforaminal involvement]. / Hemangioblastoma radicular extraforaminal de la raíz L4 derecha.

Hemangioblastomas are benign hypervascular tumours, which are frequently located at the posterior fossa or the spinal cord. Nerve root hemangioblastomas account for less than 2% of total cases. Sometimes the initial diagnosis can be inaccurate and the final diagnosis is not reached until the surgical procedure is performed. Given the high vascularisation of this particular kind of tumour, preoperative embolisation of the tumour is recommended to reduce surgical bleeding and minimise the risk of injuries to motor nerve roots. In this particular case, the patient presented with radicular pain in the right L4 nerve root territory originated by a radicular extraforaminal hemangioblastoma. After tumour devascularization and under neurophysiological control, total excision of lesion was achieved without posterior neurological deficit.