Reporte de caso: Quiste neuroentérico intradural/extramedular en paciente joven / Case report: Intradural/extramedullary neurenteric cyst in a young patient

Introducción: Los quistes neuroentéricos son lesiones congénitas, benignas, solitarias e infrecuentes del eje espinal, compuestas de tejido endodérmico heterotópico, productos del cierre incompleto del tubo neural. Se presentan, principalmente, en hombres durante la primera y segunda década de vida. Se localizan mayoritariamente en el espacio intradural/extramedular, ventralmente a la médula espinal, a nivel cervical bajo o torácico superior. La sintomatología neurológica depende de la localización del quiste. El diagnóstico presuntivo se realiza con una resonancia magnética, y el definitivo con un estudio histopatológico, llevado a cabo posterior a la exéresis completa o parcial de la muestra, con abordaje posterior, en la mayoría de los casos. Caso: Se describe el caso de una paciente de 35 años, quien tiene antecedente de haber presentado a los 14 años un quiste neuroentérico intradural/extramedular, a nivel cervial bajo. Había debutado con cervicalgia irradiada a miembros superiores e inferiores, radiculopatía, pérdida de la fuerza muscular, e hiperreflexia. Se realizó en ese momento una resonancia magnética, evidenciando una lesión ocupante de espacio en C5 y C6, la cual fue intervenida quirúrgicamente a través de una laminectomía en C5-C6 con abordaje posterior. Se realizó exéresis completa de la misma. El estudio anatomopatológico reportó quiste neuroentérico intradural/extramedular, sin atipias celulares. La evolución posoperatoria de la paciente resultó satisfactoria. Conclusión: Se describe este caso clínico, resaltando su importancia, al tratarse de lesiones sumamente infrecuentes en la literatura médica, con sintomatología poco específica, pudiendo confundirse con otras patologías, y recidivar, incluso después de haberse extraído completamente

Introduction: Neuroenteric cysts are congenital, benign, solitary, and infrequent lesions of the spinal axis, composed of heterotopic endodermic tissue, resulting from an incomplete closure of the neural tube. They mainly occur in men, during the first or second decade of life. Most of these cysts are located in the intradural/extramedullary compartment, ventrally to the spinal cord, especially at the lower cervical or upper thoracic spine. The neurological symptomatology varies depending on the location of the cyst. The presumptive diagnosis is made with magnetic resonance imaging, and the definitive diagnosis is made with a histopathological assessment, which is done after a complete or partial resection of the mass, generally with a posterior approach. Case presentation: We describe the case of a 35-year-old female patient, with the medical history of presenting an intradural/extramedullary neuroenteric cyst, located at the lower cervical level, at the age of 14. She presented cervical pain irradiated to upper and lower limbs, radicular pain, loss of muscular strength, and hyperreflexia. A magnetic resonance imaging was indicated, showing a space-occupying lesion at the C5 and C6 levels, which was surgically intervened through a posterior cervical (C5-C6) laminectomy. A complete resection of this mass was performed. The histopathological assessment reported an intradural/extramedullary neuroenteric cyst, with no cellular atypia. The patient's postoperative progress and development were satisfactory. Conclusion: The objective is to describe this case, highlighting its importance, since these lesions are extremely infrequent in the medical literature, with a non-specific symptomatology, which is why they can be confused with other pathologies, and recur, even after their complete resection.

Clinical analysis of 56 cases with surgical operation of congenital tumor in conus medullaris position / 中国医师杂志

Objective To analyze the curative effect of 56 cases with surgical operation of congenital tumor in conus medullaris position,to provide valuable reference information for the surgical operation of congenital tumor in conus medullaris position.Methods The cases were from 56 patients with congenital tumors in conus medullaris position in Xinjiang Uygur municipal people's hospital from January 2015 to February 2018.According to the classifications of congenital tumors in the conus medullaris position,surgical operation was performed on patients.The curative effect of patients with all kinds of congenital tumors in the conus medullaris position was analyzed and summarized.Results After 9 months of the follow-up:(1) There were totally 18 cases of epidermoid cysts,16 cases with obvious improvement in lower limb numbness and lower limb motor function,13 cases with the pain degree effectively relieved and 3 cases without significant improvement in early defecation function.(2) There were 15 cases of teratomas,13 cases without significant improvement in lower extremity numbness,2 cases with reduced lower extremity numbness,3 cases with reduced urination and defecation function disturbance and 1 case not recovered.There was no recurrence of tumor.(3) There were 8 lipoma patients who were cured and discharged without urination and defecation function disturbance.Life was basically self-sustaining.(4) There were 9 cases of hemangioblastoma with significant improvement in spinal cord function,5 cases without postoperative pain symptoms and 1 case with significant improvement in pain degree.(5) There were 6 cases of ependymoma,2 cases with significant reduction on limb numbness symptoms,1 case with urinary retention after surgery.There was no significant change in muscle strength of the 2 cases before and after surgery.Conclusions The clinical effect of surgical operation of congenital tumor in conus medullaris position is clear.Combined with imaging examination and attention to surgical precautions,the surgical treatment effect of congenital tumors in the conus region of the spinal cord can be effectively improved.

Clinical analysis of 56 cases with surgical operation of congenital tumor in conus medullaris position / 中国医师杂志

Objective@#To analyze the curative effect of 56 cases with surgical operation of congenital tumor in conus medullaris position, to provide valuable reference information for the surgical operation of congenital tumor in conus medullaris position.@*Methods@#The cases were from 56 patients with congenital tumors in conus medullaris position in Xinjiang Uygur municipal people's hospital from January 2015 to February 2018. According to the classifications of congenital tumors in the conus medullaris position, surgical operation was performed on patients. The curative effect of patients with all kinds of congenital tumors in the conus medullaris position was analyzed and summarized.@*Results@#After 9 months of the follow-up: ⑴ There were totally 18 cases of epidermoid cysts, 16 cases with obvious improvement in lower limb numbness and lower limb motor function, 13 cases with the pain degree effectively relieved and 3 cases without significant improvement in early defecation function. ⑵ There were 15 cases of teratomas, 13 cases without significant improvement in lower extremity numbness, 2 cases with reduced lower extremity numbness, 3 cases with reduced urination and defecation function disturbance and 1 case not recovered. There was no recurrence of tumor. ⑶ There were 8 lipoma patients who were cured and discharged without urination and defecation function disturbance. Life was basically self-sustaining. ⑷ There were 9 cases of hemangioblastoma with significant improvement in spinal cord function, 5 cases without postoperative pain symptoms and 1 case with significant improvement in pain degree. ⑸ There were 6 cases of ependymoma, 2 cases with significant reduction on limb numbness symptoms, 1 case with urinary retention after surgery. There was no significant change in muscle strength of the 2 cases before and after surgery.@*Conclusions@#The clinical effect of surgical operation of congenital tumor in conus medullaris position is clear. Combined with imaging examination and attention to surgical precautions, the surgical treatment effect of congenital tumors in the conus region of the spinal cord can be effectively improved.

Cervical Osteoblastoma: A Case Report / Osteoblastoma cervical: relato de caso

Abstract Osteoblastoma is a rare benign primary bone tumor. It accounts for 3% of benign and 1% of all primary bone tumors. The treatment goal is complete surgical resection. This treatment limits the risk of recurrence. As osteoblastoma is a highly vascular tumor, complete resection is often difficult. This report describes the case of a 19-year-old male patient who presented severe right-sided neck and shoulder pain. The computed tomography scan revealed a mass lesion on C7 compatible with osteoblastoma. Preoperative embolization and tumor resection were performed. At the 3-year follow-up, the patient had no restrictions on daily activities, and, to date, there has been no evidence of recurrence.

Resumo Osteoblastoma é um tumor primário benigno raro. Constitui cerca de 3% dos tumores benignos e 1% de todos os tumores ósseos. O objetivo do tratamento é a ressecção completa, que limita o risco de recidiva. Por se tratar de um tumor vascularizado, a ressecção completa é, muitas vezes, difícil. Os autores descrevem um caso clínico de um paciente do sexomasculino de 19 anos que apresentava queixas de cervicalgia direita com irradiação para o ombro. O exame por tomografia computadorizada indicou uma lesão em C7 compatível com osteoblastoma. O paciente foi submetido a resseção cirúrgica após embolização pré-operatória e artrodese anterior. Aos 3 anos de seguimento, o paciente encontra-se assintomático e, até a data, sem evidência de recidiva.

Diffuse midline glioma of spinal cord with H3K27M-mutation: a clinicopathological analysis / 中华病理学杂志

Objective@#To investigate the role of H3K27M mutant in spinal cord glioma, specifically the correlation between H3K27M mutation and histological grade or prognosis.@*Methods@#Twenty-four cases of paraffin-embedded spinal cord glioma tissues and clinical data were collected from November 2014 to August 2016 at the Beijing Tsinghua Changgung Hospital. There were 13 males and 11 females, and the age ranged from 3 to 66 years. All the cases were reviewed histologically, and immunohistochemical H3K27M staining and H3 gene detection were performed. The correlation between H3 gene mutation and histological grading and prognosis of spinal cord gliomas were investigated and relevant literature reviewed.@*Results@#Eleven of 24 cases showed H3K27M gene mutation, and was concordant with the result of immunohistochemistry. Gliomas in the mutant group were all high-grade gliomas with mean patients′ age of (30.0±11.5) years, and a male to female ratio of 7:4. Thirteen cases were wild-type, and these included four high-grade gliomas, with mean patients′ age (31.3±22.4) years, and a male to female ratio of 6∶7. The tumors in the mutant group were mainly located in cervical 4-7 and thoracic 11-12 segments, respectively, and the incidence of tumors in the lower thoracic segments (thoracic 11-12) was higher than that in the wild type group. Outcome data were available for all patients. The median survival of mutant group was 19.5 months, but most patients in the wild-type group were alive at the end of the follow-up period.@*Conclusion@#Gliomas of spinal cord with H3K27M mutation are high-grade and the prognosis of patients is poor.

Spinal Cord Subependymoma Surgery: A Multi-Institutional Experience

OBJECTIVE: A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas. METHODS: We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21–77) from four institutions. RESULTS: The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8–89). CONCLUSION: Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

Spinal Cord Subependymoma Surgery: A Multi-Institutional Experience

OBJECTIVE: A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas.METHODS: We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21–77) from four institutions.RESULTS: The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8–89).CONCLUSION: Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

A Long, Solitary, Rosary-Shaped Spinal Neurofibroma

STUDY DESIGN: Case report. OBJECTIVES: We report the case of a long, solitary, rosary-shaped neurofibroma that was misdiagnosed as another disease due to the patient's surgical history involving repetitive procedures and its abnormal appearance. SUMMARY OF LITERATURE REVIEW: Neurofibroma is an intradural-extramedullary spinal tumor. It is generally not difficult to diagnose due to its frequent occurrence and specific magnetic resonance imaging (MRI) findings. However, to date, neurofibromatosis stigmata and long, solitary, rosary-shaped neurofibromas have rarely been reported. MATERIALS AND METHODS: A 60-year-old woman was admitted to our hospital due to persistent pain, despite previous surgery and repetitive procedures. On physical examination, vision loss, hearing loss, skin discoloration, or subcutaneous nodules were not observed. A neurologic examination revealed normal motor and sensory function and voiding sensation. No pathologic reflexes such as the Babinski sign were observed. Previous sequential MRIs revealed intradural lesions that progressed from the thoracic vertebra 11 to the lumbar vertebra 3. She had no signs of neurofibromatosis stigmata, and the neurologic examination was unremarkable. The initial diagnosis was based on serial MRIs, which revealed a parasite infestation, a spinal cord tumor (myxopapillary-type ependymoma with hemorrhage), arachnoiditis, and vascular malformations. Total mass excision was performed, and the final diagnosis was neurofibroma. RESULTS: There were no signs of a tumor remnants or local recurrence in a 1-year follow-up MRI study. CONCLUSIONS: Although intradural spinal tumors are very rare, their clinical features are nonspecific and resemble other degenerative spinal diseases, including spinal stenosis and disc herniation. These diseases may easily be overlooked by physicians.

Microsurgical Treatment of Sporadic and von Hippel-Lindau Disease Associated Spinal Hemangioblastomas: A Single-Institution Experience

STUDY DESIGN: Retrospective cohort study. PURPOSE: To examine the clinical profile and surgical complications in patients with spinal hemangioblastomas and to evaluate the long-term outcome in them. OVERVIEW OF LITERATURE: Although considered to be histologically benign, hemangioblastomas may cause significant neurological deficits. The proportion of spinal hemangioblastomas associated with von Hippel–Lindau (VHL) disease has been estimated be 13%–59%. Preoperative neurological function correlates with postoperative neurological status. Studies have shown no difference in outcomes between sporadic and VHL-associated spinal hemangioblastomas. METHODS: This retrospective study included 14 consecutive patients treated for spinal hemangioblastomas at our institute between January 2000 and June 2013. The mean follow-up period was 5 years. Magnetic resonance imaging of the complete neuraxis was performed in all cases, and preoperative embolization was performed in two cases. RESULTS: In total, 14 patients underwent 18 surgeries, of which 15 were for spinal hemangioblastomas. Of all the patients, 86% had motor weakness and 79% presented with sensory disturbances. Preoperative McCormick functional grades were grade I in 7 (50%), grade II in 3 (21%), and grade III in 4 (29%) patients; 50% patients were diagnosed with VHL disease. All patients underwent complete resection of the tumor. Eight patients experienced deterioration in their neurological status in the immediate postoperative period; among them, five had gradual improvement. At 5-year follow-up, 11 (78.57%) patients showed good functional outcomes. CONCLUSIONS: Microsurgical excision of spinal hemangioblastomas can cause postoperative morbidity, mainly in the form of neurological deterioration. Almost half of our patients had deterioration in the McCormick grade in the immediate postoperative period. However, a complete microsurgical excision can result in good long-term functional outcomes, as most of the immediate postoperative neurological deterioration in our patients was reversible. There was no difference in the long-term functional outcomes between sporadic and VHL-associated spinal hemangioblastomas.

Virtual Preoperative Simulation for Excision of Spinal Tumors: Surgeon Processing of Medical Computer-Assisted Design Software

As medical computer-aided design (CAD) has improved, virtual 3-dimensional medical images have been gaining more easily without any special practice. These images can be applied to various clinical fields. This article illustrates virtual preoperative simulation for excision of spinal tumors using medical CAD software. The software was used directly by the surgeon. The process of virtual preoperative simulation for spinal tumor surgery was found to be not inordinately complicated. And, virtual simulation was helpful in determining surgical steps as well as understanding the surgical anatomy.

Spinal hemangioblastomas: microsurgical treatment and long-term follow-up / 肿瘤研究与临床

Objective To summarize the treatment experiences and long-term surgical efficacy of spinal hemangioblastomas.Methods The surgical experiences of 46 patients with spinal hemangioblastomas were analyzed retrospectively.All patients underwent microsurgical resection without preoperative embolization, and 52 operations were performed and 58 tumors were resected.McCormick classification was used to evaluate the function of spinal cord.The mean follow-up time was 8 years.Results Total tumor resection was achieved in 55 tumors and subtotal ones in 3.A week later, improvement was obtained in 11 cases, no changes in 29 and aggravation in 6.No patients died.During a follow-up period from 7 to 9 years, the spinal function was improved in 18 patients, remained stable in 21 and deteriorated in 7.No tumor recurrence was found.The subtotal resection affected the prognosis (P =0.08, OR =10.8, 95 ％ CI 2.1-60.8).Conclusions The spinal hemangioblastomas are highly vascularized benign tumors that can be surgically cured under microscope.Subtotal resection is a risk factor of prognosis.

Single-Stage Operation for Giant Schwannoma at the Craniocervical Junction with Minimal Laminectomy: A Case Report and Literature Review

Here we report a single-stage operation we performed on a patient with a large schwannoma that extended from the lower clivus to the cervico-thoracic junction caudally. A number of authors have previously performed multilevel laminectomy to remove giant schwannomas that extend for considerable length. This technique has caused cervical instability such as kyphosis or gooseneck deformity on several occasions. We removed the tumor with a left lateral suboccipital craniectomy with laminectomy only at C1 and without any subsequent surgery-related neurologic deficits. However, this technique requires meticulous preoperative evaluation on existence of Cerebrospinal fluid (CSF) cleft between the tumor and spinal cord on magnetic resonance imaging, of tumor origin located at the upper cervical root, and of detachment of tumor from the origin site.

Acute Paraplegia as a Result of Hemorrhagic Spinal Ependymoma Masked by Spinal Anesthesia: Case Report and Review of Literature

Ependymomas are the most common intramedullary spinal cord tumors in adults. Although a hemorrhage within spinal ependymoma on imaging studies is not uncommon, it has rarely been reported to bea cause of acute neurological deficit. In the present report, we describe a case of a 24-year-old female patient who developed acute paraplegia as a result of hemorrhagic spinal ependymoma immediately after a cesarean delivery under spinal regional anesthesia. We review the literature of hemorrhagic spinal ependymomas presenting with acute neurological deficit and discuss the most appropriate treatment for a good neurological recovery.

Acute Paraplegia as a Result of Hemorrhagic Spinal Ependymoma Masked by Spinal Anesthesia: Case Report and Review of Literature

Ependymomas are the most common intramedullary spinal cord tumors in adults. Although a hemorrhage within spinal ependymoma on imaging studies is not uncommon, it has rarely been reported to bea cause of acute neurological deficit. In the present report, we describe a case of a 24-year-old female patient who developed acute paraplegia as a result of hemorrhagic spinal ependymoma immediately after a cesarean delivery under spinal regional anesthesia. We review the literature of hemorrhagic spinal ependymomas presenting with acute neurological deficit and discuss the most appropriate treatment for a good neurological recovery.

The Use Fibrin Sealant after Spinal Intradural Tumor Surgery: Is It Necessary?

OBJECTIVE: A fibrin sealant is commonly applied after closure of an incidental or intended durotomy to reduce the complications associated with the leakage of cerebrospinal fluid. Routine usage might not be essential after closure of an intended durotomy, which has clear cut-margins. We investigated the efficacy of fibrin sealants for primary intradural spinal cord tumor surgery. METHODS: A retrospective review was performed for 231 consecutive surgically treated patients with primary intradural spinal cord tumors without extradural extension. Fibrin sealants were not used for 47 patients (group I: age, 51.57±16.75 years) and were applied to 184 patients (group II: age, 48.8±14.7 years). The surgical procedures were identical except for the use of a fibrin sealant after closure of the durotomy. The primary outcome was the occurrence of complications (wound problems, hematoma collection, infection, and neurological deterioration). The covariates were age, sex, body mass index, operation time, pre-/postoperative ambulation, number of laminectomies, and type of tumor. RESULTS: Schwannoma was the most common pathology (n=134), followed by meningioma (n=35) and ependymoma (n=31). Complications occurred in 13 patients (3 in group I and 10 in group II, p=0.73). The postoperative ambulation status (p<0.01; odds ratio, 28.8; 95% confidence interval, 6.9-120.0) and operation time (p=0.04; cutoff, 229 minutes; sensitivity, 62%; specificity, 72%) were significant factors, whereas the use of a fibrin glue was not (p=0.47). CONCLUSION: The use of a fibrin sealant might not be essential to reduce complications after surgery for primary spinal intradural tumor.

Surgical treatment and prognostic factors of cervical intramedullary spinal cord tumors / 第二军医大学学报

Objective To summarize our experience in surgical treatment of cervical intramedullary spinal cord tumors and to identify the prognostic factors associated with postoperative-neurological status. Methods The clinical data of 65 consecutive patients with cervical intramedullary spinal cord tumors undergoing operation between Jan. 2009 and Dec. 2013 were retrospectively reviewed. McCormick Scale was applied to evaluate the preoperative and postoperative neurological functions. Multivariate logistic regression analysis was used to determine the independent prognostic factors of postoperative neurological status. Results Laminectomy was performed in 61 patients and unilateral multilevel interlaminar fenestration (UMIF) was applied in 4 patients. Of all the 65 patients, 48 received total resection, 4 received subtotal resection and 13 received partial resection. The follow-up time was from 4 months to15 years. The median follow-up time was 68 months. The clinical symptoms were relieved in 42 patients after operation, maintained unchanged in 14 and aggravated in 9. The pain remission rate of the patients was the highest (82. 4%), followed by the sphincter dysfunction remission rate (68. 8%), and the sensory disturbance remission rate of the patients was the lowest (39. 6%). Based on McCormick Scale, the postoperative neurological functions was improved in 18 patients (27. 7%) and maintained unchanged in 39 (60%) , while eight patients (12. 3%) developed neurological deterioration. Multivariate logistic regression analysis revealed that good preoperative neurological function (OR=19. 87, 95% CI; 4. 10-96. 23, 7 = 0. 000)and total resection (OR=7. 40, 95% CI; 1. 34-40. 95, 7 = 0. 022) were the independent protective factors for postoperative neurological status. Conclusion Microsurgical resection is the first-line treatment for cervical intramedullary spinal cord tumors , which can achieve a satisfying outcome in most cases. Preoperative neurological status and surgical extent are significantly associated with the postoperative functional outcome of patients.

MRI Findings of Spinal Epidural Angiolipomas with Literature Review / 中国医学影像学杂志

Purpose To investigate the MRI findings and to improve the understanding of spinal epidural angiolipomas. Materials and Methods MRI findings were retrospectively analyzed in two cases of surgery and pathology proven spinal epidural angiolipomas. Relevant literatures were reviewed. Results Two cases of spinal epidural angiolipomas were located in the lumber and lower thoracic spinal canal. The tumors were elongated or spindle in shape within the epidural space, and parallel to the longitudinal axis. In both cases part of the tumors were hyperintense on T1WI and T2WI, attenuated on fat-saturated sequences with mild enhancement. Some areas were hypointenseon T1WI, hyperintenseon T2WI, unattenuated on fat-saturated sequences with apparent enhancement. Angiolipomas were classified according to the MRI features. One was type I and the other was type III. Both cases were non-infiltrative. Conclusion MRI shows the size, shape, MRI signals and the association with the adjacent structure of spinal epidural angiolipomas. The MRI classification is helpful for improving the understanding of the tumor. MRI is the best imaging modality to diagnose spinal epidural angiolipomas.

Intramedullary non-dysraphic cervical spine lipoma - case report / Lipoma intramedular da coluna cervical não disráfica - relato de caso

Intramedullary lipomas correspond to about 1% of the intramedullary tumors. These lesions are commonly associated with spinal dysraphisms and midline defects. Non-dysraphic lipomas are quite rare lesions, potentially located at any site of the spinal cord. Here we present the case history of an intramedullary non-dysraphic cervical spine lipoma.

Lipomas intramedulares correspondem a aproximadamente 1% dos tumores intramedulares. Essas lesões são comumente associadas com disrafismos e defeitos da linha média. Lipomas não disráficos são lesões muito raras, potencialmente localizadas em qualquer local da medula espinhal. Neste trabalho apresentamos o caso de um lipoma intramedular da coluna cervical não disráfica.

Estudo das alterações na expressão gênica dos ependimomas / Study of gene expression alterations in ependymomas

Ependimomas são tumores gliais raros. Podem ser encontrados em qualquer localização do sistema nervoso central e, apesar de histologia similar, parecem apresentar alterações genômicas distintas. As variáveis clínicas são intercorrelacionadas e, geralmente, incapazes de predizer o curso da doença. O objetivo do presente estudo foi analisar a expressão aumentada de genes e proteínas em ependimomas e correlacionar com dados clínicos dos pacientes. Foram estudados casos de pacientes com ependimoma submetidos à ressecção cirúrgica no Hospital das Clínicas, Universidade de São Paulo, no período entre 1996 e 2011 (33 amostras de tecido congelado para análise de expressão gênica por PCR quantitativo em tempo real e 149 amostras com tecido incluído em parafina, correspondentes a 121 casos devido a recidivas, para análise de proteína por imuno-histoquímica de tissue microarrays). As reações de imuno-histoquímica foram analisadas semiquantitativamente e graduadas com um índice de marcação calculado pelo produto da porcentagem de núcleos marcados pela intensidade de marcação. Oitenta e um casos eram adultos (média de 27,2 anos). Havia 60 casos intracranianos e 61 intramedulares, dos quais 10 eram mixopapilares, 92 grau II e 19 grau III. Ressecção completa foi possível em 62% dos casos e recidiva foi confirmada em 41,1%. Observou-se menor tempo para recidiva em crianças e tumores intracranianos, supratentoriais (p < 0,001 em ambos), histologia anaplásica e ressecções incompletas (p < 0,05 em ambos). Os seguintes genes foram selecionados em dados públicos de SAGE e literatura: ARMC3, CCND1, CHST5, DNALI1, FGFRL1, GNA13, IGF2, MSX1, NOTCH1 e RSPH3. ARMC3, RSHL3, CHST5 e DNALI1 apresentaram maiores níveis de expressão em ependimomas intramedulares (p < 0,05), e FGFRL1, NOTCH1 e CCND1 nos casos supratentoriais (p < 0,01). IGF2 apresentou maiores níveis de expressão em crianças e CHST5 em adultos (p < 0,05 em ambos). Foram observados maiores níveis de expressão de FGFRL1...

Ependymomas are rare glial cell-derived tumors. They can be found in any central nervous system localization and despite the histological similarity, they seem to display distinct genomic abnormalities. Clinical variables are intercorrelated and they are usually unable to predict the disease course. We aimed to analyze increased gene and protein expression in ependymomas and to correlate with patients' clinical data. We studied patients with ependymoma submitted to surgical resections at Hospital das Clinicas, University of São Paulo, from 1996 to 2011 (33 fresh-frozen samples for gene expression analysis by quantitative real-time PCR and 149 formalin-fixed, paraffin-embedded samples, relative to 121 patients due to relapses, for protein analysis by tissue microarray immunohistochemistry). Immunohistochemical reactions were analyzed semi-quantitatively and scored with a labeling index (LI) calculated as the product of the percentage of the positively stained nuclei by the intensity of staining. Eighty-one cases were adults (mean 27.2 years). There were 60 intracranial and 61 spinal cases, of which 10 tumors were myxopapillary, 92 were grade II and 19 were grade III. Gross total resection was achieved in 62% of cases and relapse was confirmed in 41.4% of cases. We observed a shorther time to relapse in children and supratentorial intracranial tumor localization (p<0.001 for both), anaplastic histology and incomplete resections (p<0.05 for both). The following genes were selected based on public SAGE database and literature: ARMC3, CCND1, CHST5, DNALI1, FGFRL1, GNA13, IGF2, MSX1, NOTCH1 and RSPH3. ARMC3, RSHL3, CHST5 and DNALI1 presented higher expression levels in intramedullary ependymomas (p < 0.05) and FGFRL1, NOTCH1 and CCND1 in supratentorial cases (p < 0.01). IGF2 presented higher expression levels in pediatric cases and CHST5 in adults cases (p < 0.05 in both). Higher expression levels of FGFRLI1 (p < 0.05), CCND1 and IGF2...