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.

Mortality is higher in patients with leptomeningeal metastasis in spinal cord tumors / Mortalidade é mais elevada na disseminação metastática leptomeníngea em tumores da medula espinhal

Spinal cord tumors are a rare neoplasm of the central nervous system (CNS). The occurrence of metastases is related to poor prognosis. The authors analyzed one series of metastasis cases and their associated mortality. METHODS: Clinical characteristics were studied in six patients with intramedullary tumors with metastases in a series of 71 surgical cases. RESULTS: Five patients had ependymomas of which two were WHO grade III. The patient with astrocytoma had a grade II histopathological classification. Two patients required shunts for hydrocephalus. The survival curve showed a higher mortality than the general group of patients with no metastases in the CNS (p<0.0001). CONCLUSION: Mortality is elevated in patients with metastasis and greater than in patients with only primary lesions. The ependymomas, regardless of their degree of anaplasia, are more likely to cause metastasis than spinal cord astrocytomas.

Tumores da medula espinhal são neoplasias raras do sistema nervoso central (SNC). A ocorrência de metástases é relacionada a pior prognóstico. Os autores analisaram uma série de casos de metástases e a mortalidade relacionada. MÉTODO: Foram estudadas as características clínicas em seis pacientes com metástases tumorais numa série de 71 casos operados. RESULTADOS: Cinco pacientes tinham ependimomas e dois dos quais foram grau III pela classificação da OMS. O paciente portador de astrocitoma tinha classificação histopatológica de grau II. Dois pacientes necessitaram de derivação devido à hidrocefalia. A curva de sobrevivência mostrou mortalidade mais elevada no grupo de pacientes com disseminação pelo SNC (p<0,0001). CONCLUSÃO: A mortalidade, além de elevada em pacientes com metástases, é maior do que em pacientes apenas com lesão primária. Os ependimomas, independentemente do seu grau de anaplasia, costumam causar mais metástases do que os astrocitomas medulares.

Intramedullary Spinal Cord Metastasis in Renal Cell Carcinoma: A Case Report of the Surgical Experience

Intramedullary spinal cord metastasis (ISCM) from renal cell carcinoma (RCC) is rare manifestation and most of them are treated by adjuvant treatment modalities like radiotherapy. Despite the radio-resistance of RCC itself, focal radiotherapy has been preferred as the first-line treatment modality of ISCM from RCC and only a few cases underwent surgical treatment. We describe a case of ISCM from RCC, which underwent surgical excision and pathologically confirmed. A 44-yr-old man was presented with rapid deterioration of motor weakness during focal radiotherapy for ISCM from RCC. After the surgery for removal of the tumor mass and spinal cord decompression, his motor power was dramatically improved to ambulate by himself. We report the first published Korean case of ISCM from RCC confirmed pathologically and describe our surgical experience and his clinical characteristics.

Cervical Intramedullary Ancient Schwannoma

A young male of 24 years was presented with a history of gradually progressive spastic quadriparesis for long four years. He was investigated and MRI revealed a neoplastic lesion in the cervical cord at the level of C4, C5 with perilesional edema and tumour syrinx formation, suggestive of astrocytoma. After total removal histopathology proved it to be an ancient schwannoma.

Schwannoma melanótico: reporte de un caso y revisión de la literatura / Melanotic schwannoma: a case report and literature review

Schwannomas are benign neoplastic lesions which originate from Schwann cells. A rare variant is the melanotic schwannoma. Accurate discrimination of this entity may be difficult due to differential diagnosis with malignant tumors, especially with metastatic melanoma, which has a potential ominous prognosis and a radically different treatment. We report the case of a 60-year-old woman with neurological sensorimotor involvement, presenting progressive caudo-cranial involvement more pronounced on the right side. MRI showed an intradural extramedullary mass with hyperintense signal intensity on T1 and T2 images, whereas no significant increase in signal intensity of the spinal cord was observed. Tumor was resected and sent for anatomopathological analysis which revealed a non-psammomatous melanotic schwannoma.

Los schwannomas son lesiones neoplásicas benignas derivadas de las células de Schwann. Una variante poco frecuente es el schwannoma melanótico. El diagnóstico de esta entidad reviste complejidad debido al diagnóstico diferencial con tumores malignos, especialmente el melanoma metastásico, teniendo este último un potencial pronóstico ominoso y un tratamiento radicalmente distinto. Se realizó una revisión de literatura en relación a un caso clínico de una mujer de 60 años con compromiso neurológico de tipo sensitivo-motor, progresivo caudo-craneal mayor a derecha. La RM demostró una masa intradural extramedular, con hiperintensidad en secuencias T1 e hiposeñal en T2, sin aumento de señal significativo de la médula espinal. El tumor fue resecado revelando en el estudio anatomopatológico un schwannoma melanótico, no psammomatoso. En relación al caso clínico, pudimos observar la importancia del estudio imaginológico con RM y su confirmación anatomopatológica. Recalcamos además, la necesidad del seguimiento a largo plazo.

spectrum of intradural spinal tumors

To assess the spectrum of clinical, radiological and histological features of patients with intradural spinal tumors. This descriptive study was carried out in Department of Neurosurgery Lady Reading Hospital Peshawar, from April 2003 to March 2009. Medical records of patients with spinal tumors were reviewed and patients operated for intradural spinal tumors were identified. A total of 312 patients, out of 525 cases of spinal tumors, with different intradural spinal tumors were considered in this study. Their clinical features, radiological reports, peroperative findings and histological reports were analyzed in different aspects. There were total of 312 patients with age range from 2 years to 74 years, with median age of 38 years. Out of these 187 were males and 125 were female, overall male to female ratio of 1.5: 1. Backache, leg weakness, parasthesia and poor sphincters were the main clinical features. MRI spine [274 cases] was the main diagnostic tool along with plain X-rays and X-ray myelography in limited cases [35 cases]. CT myelogram was done only in 3 cases. The common site of involvement was dorsal spine followed by lumber and cervical spines respectively in 185, 80 and 47 cases. Histological report was suggestive of Neurofibroma in 166 Meningioma in 96, Ependymoma in 20, Dermoid in 12, Astrocytoma in 7, Hemangioblastom and Tuberculoma in 3 cases each and Hydatid cyst in one case. Neurofibroma and meningioma constituted majority of cases belonging to intradural extramedulary group, while ependymoma and astrocytoma were common intramedullary tumors. Third and 5[th] decade of life was the common age group for both Intramedulary and extramedulary tumors. Intramedulary lesions were common in 3[rd] decade of life

Experience with 349 cases of intradural spinal tumors at Lady Reading Hospital Peshawar

To analyze the variables of patients operated for intradural spinal tumors at Lady Reading Hospital Peshawar. An analytical descriptive study Place and duration of study: Department of Neurosurgery Lady Reading Hospital Peshawar, from April 2003 to March 2010 [7 years]. Medical record of patients with spinal tumors were revised and patients suffering from intradural spinal tumors were searched. There were total 572 cases of spinal tumors, of which 349 had different intradural lesions. Their clinical features, radiological reports, peroperative findings and histological reports were analyzed in different aspects. Of the total 349 patients with intradural spinal tumors 201 were males and 148 female, with male to female ratio of almost 1.4:1. Age of the patients ranged between 2 years to 77 years, with median of 39.2 years. The common clinical features were backache, leg weakness, parasthesia and poor sphincters. Magnetic Resonance Imaging of the spine was the main [309 cases] diagnostic tool along with plain x-ray and myelography in limited cases [35 cases]. CT myelogram was done only in 5 cases. Dorsal spine was involved in 62.5% cases, lumber and cervical spine in 25.5% and 12% patients respectively. Neurofibroma was the commonest [51%] intradural spinal tumor, we also had meningioma in 30.66%. Other intraspinal lesions were dermoid, lipoma, ependymoma, astrocytoma, tuberculoma, arachnoid and hydatid cysts. Of all the spinal tumors intradural are more common [61%] than extradural. Neurofibroma and meningioma constituted majority of cases belonging to extramedullary intradural group, while ependymoma and dermoid were common intramedullary tumor. 3rd and 5th decade of life were the common age group for both intramedulary and extramedullary tumors. Intramedullary tumors were common in 3rd decade of life

Surgical management of intramedullary spinal ependymomas / Abordagem cirúrgica dos ependimomas intramedulares

BACKGROUND: Spinal intramedullary ependymoma is a rare disease with a wide range of clinical presentation, generally requiring surgical treatment. OBJECTIVE: Report our experience and present our surgical technique to achieve total resection and cure. METHOD: We present 12 consecutive cases of intramedullary ependymomas operated between 2000 and 2008 by the senior author (HT). The functional scale proposed by McCormick was used to evaluate the patients' neurological status. RESULTS: Age at presentation varied from 18 to 55 (average 36) years. All tumors had a benign histology. Four (33 percent) patients were male and eight (67 percent) were female. According to the site of presentation, six (50 percent) were localized at the cervical region (including two at the cervicomedullary junction, two at the cervico-thoracic junction and two exclusively at the cervical level), four at the thoracic level and two at the conus/ cauda equina. Dyshestesia was a common finding at the neurological exam in eight patients (67 percent). Total resection was achieved in all cases. Six patients showed neurological improvement postoperatively, whereas the other six remained stable. CONCLUSION: Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors with minimal morbidity and maximum functional recovery.

INTRODUÇÃO: Os ependimomas intramedulares são lesões raramente encontradas na prática neurocirúrgica, tendo apresentação clínica variada, geralmente requerendo tratamento cirúrgico. OBJETIVO: Relatar nossa experiência e discutir a técnica microcirúrgica para a ressecção total e conseqüente cura destas lesões. MÉTODO: Apresentamos uma série de 12 casos de ependimomas intramedulares operados sucessivamente entre 2000 e 2008 pelo autor sênior (HT). A evolução neurológica foi avaliada através da classificação funcional de McCormick. RESULTADOS: A idade dos pacientes variou de 18 a 55 anos (média de 36 anos). Todos eram histologicamente benignos. Quatro (33 por cento) eram do sexo masculino e oito do feminino (67 por cento). A localização das lesões esteve distribuída da seguinte forma: seis casos (50 por cento) na região cervical (sendo dois na transição cérvico-bulbar, dois na região cérvico-torácica e dois na região cervical isoladamente), quatro na região torácica e dois no nível do conus-cauda-eqüina. Disestesias estavam presentes em oito pacientes no pré-operatório (67 por cento dos casos). A ressecção total foi atingida em todos os casos. Em seis casos (50 por cento), houve melhora dos sintomas neurológicos, enquanto que nos outros seis houve manutenção do quadro clínico. CONCLUSÃO: O conhecimento anatômico e de técnicas microcirúrgicas adequadas permite a ressecção total destas lesões propiciando a cura da doença com mínima morbidade e máxima recuperação funcional.

Hemangioma vertebral del arco posterior con extensión extraósea y sintomatología neurológica: informe de un caso y revisión de la literatura / Vertebral hemangioma of the posterior arch with subsequent extraosseous extension and neurological symptoms: case report and literature review

Introducción: El hemangioma vertebral es el tumor más común de la columna vertebral, se identifica hasta en 11 % de las autopsias. Es tipificado como una malformación benigna vascular de crecimiento lento; en ocasiones involucra los elementos posteriores, asociándose a compromiso neurológico. Por lo general es asintomático, encontrándose de forma incidental al estudiar el dolor de origen espinal en busca de otra patología; ocasionalmente produce manifestación neurológica. La extensión extraósea intrarraquídea con compromiso neurológico ha sido poco informada y representa un reto terapéutico. Caso clínico: Adolescente femenina con hemangioma vertebral de tamaño inusual del arco posterior con extensión hacia el conducto raquídeo que ocasionó manifestaciones neurológicas y ameritó tratamiento quirúrgico para su resección y fijación transpedicular. Conclusiones: El hemangioma vertebral es una lesión asintomática que no requiere tratamiento específico. Solo en algunos casos es necesario el tratamiento quirúrgico, que debe llevarse a cabo oportunamente cuando hay compresión neurológica, para evitar secuelas permanentes. El crecimiento extraóseo intrarraquídeo con deterioro neurológico es una presentación inusitada.

BACKGROUND: Vertebral hemangioma is the most common benign spinal tumor and is found in 11% of postmortem studies as a slow-growing benign vascular malformation. It usually involves the vertebral body and sometimes the posterior vertebral elements. When the posterior elements are involved, spinal cord compression with neurological symptoms are seen more often. Vertebral hemangiomas exist as a continuum of manifestations and lesions ranging from the common asymptomatic forms to the rare compression lesion. Extraosseous extension of vertebral hemangioma with cord compression and neurological symptoms is a rare condition and represents a treatment challenge. Surgical options are open resection, embolization or vertebroplasty. CLINICAL CASE: We report a case of a vertebral hemangioma with extraosseous extension to the spinal canal with an unusual size and osseous component in the posterior elements. This tumor caused neurological manifestations and was surgically treated with posterior decompression and stabilization with transpedicular screws and rods. Clinical outcome was good. CONCLUSIONS: Vertebral hemangioma is normally an asymptomatic benign lesion not requiring specific treatment. Only in a few cases is surgical treatment required. When neurological compromise is present, early treatment should be carried out before the presence of permanent paralysis.

Tumores intradurales extramedulares primarios tratados en el Instituto Nacional de Rehabilitación / Primary intradural extramedullary tumors treated at the National Institute of Rehabilitation (Mexico)

Introducción: Los tumores de la columna son lesiones poco comunes que afectan a una porción menor de la población, sin embargo, pueden causar morbilidad significativa y estar asociados a mortalidad. Representan 15 % de los tumores craneoespinales. El objetivo del presente trabajo fue definir la frecuencia de tumores intradurales extramedulares de columna vertebral en el Servicio de Cirugía de Columna Vertebral del Instituto Nacional de Rehabilitación, y su diagnóstico histopatológico definitivo. Material y métodos: Estudio retrospectivo de una serie de pacientes tratados quirúrgicamente entre 1996 y 2006 por diagnóstico de tumor intradural extramedular. Se evaluó sexo, edad, localización, sintomatología y tipo de tumor. Resultados: Se revisaron 27 pacientes, 11 hombres y 16 mujeres, con edad promedio de 47.33 años. Los síntomas principales fueron dolor y pérdida de la fuerza. La localización más frecuente fue la torácica seguida de la lumbar; el diagnóstico histopatológico indicó meningioma en 12 casos, schwannoma en 12 y neurofibroma en tres. Conclusiones: La sintomatología, localización y predominio de sexo son similares a los informados en la literatura. A diferencia de otras series, los tumores más frecuentes en la nuestra fueron schwannomas y meningiomas. Es importante el diagnóstico oportuno y tratamiento adecuado, que en nuestros pacientes consistió en resección total con laminoplastia, para evitar secuelas neurológicas permanentes.

BACKGROUND: Spine tumors are uncommon injuries affecting a low percentage of the population; however, these tumors can cause significant morbidity and may be related to mortality. They represent 15% of craniospinal tumors. The objective of this study is to determine the frequency of intradural extramedullary spine tumors treated in our Service and to report the definitive histopathological diagnosis. METHODS: This was a retrospective study in a series of patients treated surgically with a diagnosis of intradural extramedullary tumor in the Spine Service of the National Rehabilitation Institute (Mexico) from 1996 to 2006. The following variables were evaluated: gender, age, tumor localization, symptomatology and type of tumor. RESULTS: Files of 27 patients were reviewed. There were 11 men and 16 women with an average age of 47.33 years. The main symptoms were pain and motor weakness. The most frequent localization was thoracic followed by lumbar. Histopathological diagnosis reported 12 cases of meningioma, 12 cases of schwannoma and 3 cases of neurofibroma. CONCLUSIONS: Symptomatology, localization and gender predominance are similar to that reported in the literature. Different from other series, schwannomas and meningiomas were the most frequent tumors in our study. Appropriate diagnosis and opportune treatment consisting of total resection with laminoplasty are crucial in order to avoid permanent neurological consequences.

Intradural neurenteric cyst of the cervical spine

A 60-year-old man presented with an intradural extramedullary neurenteric cyst of the cervical spinal cord. He had acute neurological deterioration in the form of quadriparesis and urinary incontinence. The patient had a history of paraneoplstic myasthenia 3 years prior to this admission. The clinical, radiological, and surgical findings are discussed together with a brief critical review of the literature. The patient had no other association anomalies. The cyst was removed totally with good neurological recovery. The clinical presentation was rather atypical for neurenteric cyst. Neurenteric cyst should be considered in the differential diagnosis of an intradural mass lesion regardless of the age, clinical presentation or location of the lesion

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.

[ case of spinal immature teratoma]

We report a rare case of intradural immature teratoma in 2-year-old girl, interesting conus medullaris to sacrum, worsening neurological deficits. The neoplasm discovered by magnetic resonance imaging was completely resected. We describe the clinical, radiological and pathological findings of this tumor with a review of the Literature and we insist in the difficulty of treatment

A Dumbbell-Shaped Solitary Fibrous Tumor of the Cervical Spinal Cord

A 40-year-old Asian female presented with a 2-month history of right shoulder pain and right triceps weakness. MRI revealed an extramedullary, extradural, dumbbell-shaped spinal cord tumor with C6 to C7 iso- and hyperintensity on T1 and T2 weighted imaging, respectively. Histological examination revealed monomorphous spindle cells with a storiform pattern. Immunohistochemistry was positive for CD34, CD99, and negative for EMA, SMA, and S100; solitary fibrous tumor (SFT) was confirmed.

Imaging features and treatment of an intradural lumbar cystic schwannoma

O schwannoma cístico intra-espinal é tumor muito raro e poucos casos estão descritos na literatura: são usualmente assintomáticos e somente diagnosticados quando atingem grande tamanho causando compressão radicular. Com o intuito de ilustrar as armadilhas existentes referentes ao diagnóstico e tratamento desse tipo de tumor, nós relatamos um caso raro, focando nos passos da investigação e terapêutica. É descrito o caso de um paciente de 55 anos que apresentava apenas queixas de dor lombar. A investigação revelou uma lesão cística extensa na região intradural lombar inferior. O tumor foi totalmente ressecado por técnica microneurocirúrgica, sendo a dura-máter reconstruída. O diagnóstico patológico e imuno-histoquímico evidenciou tratar-se de um schwannoma cístico. Neste artigo, nós procuramos enfatizar as características clínicas e tratamento de schwannomas lombares, ilustrando os achados imagenológicos desse caso incomum.