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

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

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

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

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

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

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.

Asociación entre diastematomielia y meduloepitelioma: reporte de un caso y revisión de la literatura / Association between diastematomyelia and medullo epithelioma: case report and literature review

Aim: Case report of association between diastematomyelia and medulloepithelioma. Method: 14-year-old patient with lower back pain and recent neurological deficit in extremities. CT and MRI scans of the thoracolumbar spine revealed a diastematomyelia. Intraoperative examination confirmed the presence of a spinal dysraphism and associated tumor, which was almost completely resected. Result: Histopathological and immunohistochemical findings were consistent with medulloepithelioma. Her postoperative course was uneventful. Conclusion: Diastematomyelia may manifest during adolescence as lower back pain and neurological deficit. The association of this malformation with a neoplasia is extremely rare; the present case describes concomitance with medulloepithelioma.

Reporte de un caso de asociación de diastematomielia y meduloepitelioma. Método: Paciente de 14 años con dolor lumbar y déficit neurológico en extremidades de reciente instalación. En TAC y RNM de columna dorsolumbar se pesquisa una diastematomielia. La exploración intraoperatoria, comprueba la presencia de una disrafia espinal y un tumor asociado al defecto, que se reseca casi en su totalidad. Resultado: Los hallazgos histopatológicos e inmunohistoquímicos son concordante con meduloepitelioma. La paciente tuvo un postoperatorio satisfactorio. Conclusión: La diastematomielia se puede manifestar durante la adolescencia como dolor lumbar y déficit neurológico. La asociación de esta malformación, con una neoplasia es muy poco frecuente, el presente caso describe la concomitancia con un meduloepitelioma.

Angioma capilar espinal intradural: reporte de un caso y revisión de la literatura / Spinal cord capillary hemangioma: a case report and literature review

INTRODUCTION: Capillary angiomas are extremely rare in the spinal intradural space (being even less frequent in the intramedullary location)10-24. We analyze the characteristics of these lesions. METHODS: We present a case report of a patient with a symptomatic spinal-intradural capillary hemangioma at the thoracic level, followed by a review of the literature. RESULTS: We found a total of 41 patients reported with single angiomas, and three more patients with multiple lesions. Most patients were male (33/44 patients), and the mean age was 53,5 years. The localization of these lesions was mainly in the dorsal spine (24), cauda equina (15) and conus medullaris (6), with one case reported in the cervical spine 11. Most patients presented with back pain, mielopathy or radiculopathy. Contrary to the case of cavernous angiomas, we did not find cases of capillary angiomas presenting with subarachnoid or intramedullary hemorrhage. The imaging characteristics (isointense at T1WI, hyperintense at T2WI and with intense contrast enhancement) were constant in almost all the reports. CONCLUSIONS: Spinal intradural capillary angiomas are rare vascular lesions, frequently mistaken for intradural tumors. It affects mostly males in the fifth or sixth decade of life, and is preferentially located in the thoracolumbar spine. It can be occasionally associated with marked spinal cord edema, specially when there is a intramedullary component. These lesions have a good prognosis after surgical treatment, and must be taken into account in the differential diagnosis of intradural tumors of thoracolumbar spine and cauda equina.

Primary intraspinal ganglioneuroblastoma of the thoracic spine: A rare case report.

Cerebral ganglioneuroblastoma is an embryonal tumor of the central nervous system, which has been rarely encountered into the spinal cord. The standard treatment for ganglioneuroblastoma is complete surgical excision. A 15-year old boy was presented with cord compression. Magnetic resonance imaging revealed an intradural and intramedullar enhancing lesion over T2 spine. A histomorphological diagnosis was made in the presence of immature small round cells admixed with a good number of ganglion cells. The morphological diagnosis was verified by immunohistochemistry. This is the first reported case of compressive myelopathy in the thoracic region of the spine.

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.

Spinal neurofibroma masquerading as a herniated disc. A case report

We present the only case in English medical literature of a spinal neurofibroma misdiagnosed as a herniated disc using magnetic resonance imaging [MRI]. This case presented with typical symptoms and radiological findings of a herniated disc. Intraoperatively, an abnormality was noted at the S1 nerve root sleeve. Further exploration revealed a spinal neurofibroma which was completely resected, resulting in an improvement in the patient's symptoms. Currently, there is heavy reliance on MRI as a highly sensitive and specific tool used in the diagnosis of herniated lumbar discs. Although there have been occasional reports of misdiagnoses using MRI, there are no reported cases of a spinal neurofibroma being misdiagnosed as a herniated lumbar disc. Despite great advances in radiological diagnostic imaging, surgical surprises do still occur. Ultimately, instinct is still essential in intraoperative surgical decisions

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.

Spinal Cord Glioblastoma Induced by Radiation Therapy of Nasopharyngeal Rhabdomyosarcoma with MRI Findings: Case Report

Radiation-induced spinal cord gliomas are extremely rare. Since the first case was reported in 1980, only six additional cases have been reported.; The radiation-induced gliomas were related to the treatment of Hodgkin's lymphoma, thyroid cancer, and medullomyoblastoma, and to multiple chest fluoroscopic examinations in pulmonary tuberculosis patient. We report a case of radiation-induced spinal cord glioblastoma developed in a 17-year-old girl after a 13-year latency period following radiotherapy for nasopharyngeal rhabdomyosarcoma. MRI findings of our case are described.

Diagnosis of, surgical technique for and treatment results from medullary lipomas associated with spinal dysraphism: experience with 38 patients / Diagnóstico, técnica cirúrgica e resultados nos lipomas medulares associados ao disrafismo vertebral: experiência com 38 pacientes

OBJECTIVE: To observe whether microsurgical removal of medullary lipomas and untethering of the medulla is a safe and efficient procedure. METHOD: A retrospective study was carried out on 38 patients with medullary lipomas associated with spinal dysraphism who underwent operations between January 1986 and January 2008, at the Neurosurgery Department of the Federal Hospital for State Public Servants, in Rio de Janeiro. RESULTS: No deaths occurred in this series, and there was no worsening of motor or bladder function among the patients. Seven individuals presented improvements in their motor deficit. Nine patients presented improvements in bladder function. Three individuals with trophic lesions achieved wound healing. CONCLUSION: Microsurgical removal of medullary lipomas associated with spinal dysraphism proved to be a safe procedure without deaths and with a low morbidity rate, and several patients achieved improvements in their neurological symptoms.

OBJETIVO: Observar se a remoção microcirúrgica dos lipomas medulares e a liberação da medula da tração exercida pelo lipoma é um procedimento seguro e eficaz. MÉTODO: Realizamos estudo retrospectivo de 38 pacientes com lipomas medulares associados ao disrafismo espinhal operados entre janeiro de 1986 a dezembro de 2009 no Serviço de Neurocirurgia do Hospital Federal dos Servidores do Estado do Rio de Janeiro. RESULTADOS: Nessa série não ocorreu nenhum óbito, ou piora da função motora ou vesical em nenhum paciente. Observamos melhora do défice motor em 7 pacientes. Nove pacientes apresentaram melhora da função vesical. Três indivíduos com lesões tróficas apresentaram cicatrização das suas feridas. CONCLUSÃO: A remoção microcirúrgica dos lipomas medulares associados ao disrafismo espinhal se mostrou segura, sem nenhum óbito, com baixa morbidade e com melhora dos sintomas neurológicos em vários pacientes.

A 10 year unsuspected lumbar schwannoma: a case report / Un schwannoma lumbar insospechado por 10 años: reporte de un caso

One case of unsuspected schwannoma is reported in a 69-year old man with chronic lumbosacral pain. The tumour had been mistaken for a lumbar lipoma for a decade. The definitive diagnosis was established after surgery based on the characteristic histopathological findings. The purpose of this report is to alert primary care physicians to peripheral sheath nerve tumours, in addition to highlight the role of pain and image features in the suspicion of tumours with longterm evolution.

Se relata un caso de schwannoma insospechado en un hombre con dolor lumbosacral crónico. El tumour había estado equivocado por el lipoma lumbar durante una década. El diagnóstico definitivo puede establecerse después de la cirugía, con base en los resultados histopatológicos característicos. El propósito de este informe es dar énfasis al conocimiento de médicos del cuidado primario sobre los tumoures de la vaina de nervios periféricos, y resaltar el papel del dolor e de las imágenes en la sospecha de estos tumoures que evolucionan con equívocos diagnósticos a largo plazo.

Intradural Spinal Lipoma without dysraphism

Intradural lipomas are rare tumors of the spinal canal. The aim of this paper is to report four cases of an unusually late presentation of a young male with this tumor

A Spinal Cord Astrocytoma and Its Concurrent Osteoblastic Metastases at the Time of the Initial Diagnosis: a Case Report and Literature Review

Bone metastasis from a spinal cord astrocytoma has been reported only twice in the English medical literature. It is generally known that bone metastasis is found after the initial diagnosis with/without intervening surgery rather than being found at the time of the diagnosis of astrocytoma. The purpose of this article is to report for the first time a case of concurrent bone metastasis from a spinal cord astrocytoma at the time of diagnosing the spinal cord astrocytoma.

Intramedullary tumors in children: analysis of 24 operated cases / Tumores intramedulares em crianças: análise de 24 casos operados

Intramedullary tumors are rare. The authors reviewed 24 cases operated between 1996 and 2006. The study assessed the clinical characteristics and surgical results based upon the neurological function. METHOD: Medical records of patients with intramedullary astrocytoma and ependymoma were reviewed. The minimal follow up time was 6 months and, at the end of this period, a comparative analysis of the neurological function was performed based using the McCormick scale score. RESULTS: Most patients had astrocytoma (75 percent). Male gender was more prevalent (58.3 percent). The most common type of tumor was graded as I or II, and in three cases these were malignant. The total resection of the tumor was achieved in 20.8 percent of the cases. The statistical analysis did not show a statistically significant difference between preoperative and postoperative grades at McCormick scale. CONCLUSION: The authors concluded that microsurgery to intramedullary tumors did not significantly alter the neurological function after six months.

Os tumores intramedulares são doenças raras. Os autores analisaram 24 casos operados entre 1996 e 2006. O estudo analisou as características clínicas e o resultado da cirurgia quanto à função neurológica. MÉTODO: Foram analisados pacientes com astrocitomas e ependimomas intramedulares. O tempo mínimo de acompanhamento foi de 6 meses e ao final deste período foi realizada a avaliação comparativa da variação do estado neurológico baseado na escala de McCormick. RESULTADOS: A maioria dos pacientes era de astrocitoma (75 por cento). O gênero masculino foi mais prevalente (58,3 por cento). A maioria dos tumores era de grau I ou II, 3 casos eram malignos. A ressecção total do tumor ocorreu em 20,8 por cento dos casos. A avaliação estatística demonstrou que não houve diferença significativa entre o estado neurológico na escala de McCormick pré-operatória e pós-operatória. CONCLUSÕES: Os autores concluem que a microcirurgia para ressecção dos tumores intramedulares não ocasionou variação funcional significativa nos pacientes após seis meses da cirurgia.

Subependymoma of the spine

Subependymoma is a rare benign CNS tumor [WHO grade I]. The common sites of origin for this type of tumor are the lateral and the fourth ventricles. A spinal intramedullary location is rare. Radiological features of intramedullary subependymoma can mimic more common and more aggressive tumor types. We report a case of a 61-year-old male who presented with a 3-year history of low back pain, associated with right lower limb progressive weakness and sensory numbness. An MRI of the lumbar spine revealed an intradural intramedullary lesion extending from the level of T10-L1. Clinical presentation, radiological, and pathological studies of this case of subependymoma are presented. The incidence of spinal subependymoma was also discussed in light of a literature review

Melanocitoma meníngeo intramedular dorsal / Thoracic spinal cord meningeal melanocytoma

O melanocitoma meníngeo é uma lesão infrequente, benigna, mas localmente agressiva. Sua localização intramedular é muito rara, existindo apenas três casos descritos na literatura. A propósito deste caso clínico, os autores reveem a literatura e discutem as características clínicas, imagiológicas e histológicas desse tipo de lesão. Relato do caso: Homem de 54 anos de idade com melanocitoma meníngeo intramedular dorsal (T11). Clinicamente apresentou, de início, disestesias torácicas, sem déficits neurológicos associados, que se mantiveram estáveis ao longo de dois anos. Após esse período teve instalação súbita de paraparesia. O diagnóstico definitivo foi estabelecido por exame histológico e imunocitoquímico após ressecção cirúrgica parcial da lesão. A pesquisa de melanoma maligno cutâneo--mucoso ou ocular foi negativa. Apesar de evolução inicial favorável apresentou recrescimento tumoral dois anos e meio após a primeira cirurgia, sendo reoperado. Apesar da ausência de complicações diretamente relacionadas com a cirurgia, acabou por falecer por sepsis na sequência de ruptura de diverticulite intestinal semanas depois.

Meningeal melanocytomas are infrequent, benign tumors but with a locally agressive behaviour. Extension to the spinal cord of these lesions is very rare and there are only three cases reported in the literature. The authors review the literature and discuss the clinical, neuroimaging and pathological features of this lesion. Case report: The authors present a case of a 54-year-old man with a thoracic spinal cord meningeal melanocytoma (T11). During two years the only complain was thoracic disestesias with no neurological deficit. After that period there was a sudden onset of paraparesia. The definitive diagnosis was made by histologic and immunocytochemical studies after parcial surgical excision of the lesion. No evidence of cutaneous, mucous or ocular malignant melanoma was found. Although an inicial favourable evolution, two and a half years after the first surgery the tumor regrowth and the patient was reoperated. No complications related to the surgery occurred but the patient had a rupture of an intestinal diverticulitis and died from sepsis some weeks later.