Neurological outcome after resection of intramedullary spinal cord tumors in children

Objective With modern surgical advances, radical resection of pediatric intramedullary spinal cord tumors (IMSCT) can be achieved with preservation of long-term neurological function. Clinical and radiographic risk factors predictive of postoperative neurological outcome may serve as a guide for surgical risk stratification. Materials and methods We prospectively reviewed the outcomes of 16 consecutive cases of pediatric IMSCT resection at a single institution. Clinical, radiographic, and operative variables were analyzed as predictors of postoperative neurological function defined by the modified McCormick score (MMS). Results Sixteen children 10 ± 5 years old presented with median (interquartile range) MMS score of 2 (1–2) with IMSCTs (eight cervical, eight thoracic) involving 4 ± 2 levels. Pathology revealed astrocytoma in 12 cases (three pilocytic, four grade II, three gradeIII, two GBM), gangliogliomas in two, ependymoma in one, and gliosis in one case. At median follow-up of 7 months, six (38%) patients experienced improved neurological function, eight (50%) remained stable, one (6%) experienced a delayed decrease in neurological function (GBM progression), and one (6%) died (GBM progression). Five (31%) patients developed persistent dysesthetic symptoms. Four (80%) patients with cystic tumors experienced neurological improvement compared to only two (18%) patients with noncystic tumors, p < 0.05. Preoperative steroid use (odds ratio, OR [95% confidence interval, CI] = 18.0 [1.24–260.1], p = 0.03) and cystic tumor (OR [95%CI] = 18.0 [1.24–260.1], p = 0.03) predicted neurological improvement after surgery. Conclusion Radical resection of pediatric IMSCTs can be achieved with low incidence of neurological injury. Sensory syndromes frequently occur after pediatric IMSCT resection and frequently affect patient’s quality of life. Tumors with compressive cysts may identify patients more likely to experience improved neurological function after surgical resection.     

脊髓髓内肿瘤的显微外科治疗

目的 总结脊髓髓内肿瘤的治疗经验.方法 回顾性分析36例经显微手术治疗的脊髓髓内肿瘤病人的临床资料,术中超声辅助定位5例.结果 室管膜瘤17例,低级别星形细胞瘤(WHO I ～II级)8例,高级别星形细胞瘤(WHO Ⅲ～Ⅳ级)3例,血管母细胞瘤3例,海绵状血管瘤2例,脂肪瘤、转移瘤、畸胎瘤各1例.室管膜瘤全切率82.4%,低级别星形细胞瘤为50.0%,高级别星形细胞瘤为33.3%,血管母细胞瘤、海绵状血管瘤和转移瘤均全切,脂肪瘤和畸胎瘤次全切.术后随访3个月,根据McCormick脊髓功能状态分级:I级23例,II级7例,Ⅲ级3例,Ⅳ级3例.结论 显微手术切除脊髓髓内肿瘤是目前最有效的治疗措施.术中超声有助于术中肿瘤定位,减少手术创伤.     

高颈段椎管内肿瘤的显微外科治疗

目的分析高位颈段椎管内肿瘤病例特点，探讨其手术方法和疗效。方法22例高位颈段椎管内肿瘤中，位于髓内者5例．位于髓外硬脊膜下者17例，其中有11例呈哑铃形生长。全部患者均行显微神经外科手术治疗，对于颈髓受压较严重者手术后采用大剂量甲基强的松龙治疗。结果本组5例髓内肿瘤中，3例室管膜瘤全切除，1例星形细胞瘤行部分切除，另1例星形细胞瘤行椎板内减压后活检。17例髓外硬脊膜下肿瘤中，有15例均在显微镜下全切除，有2例行大部切除；其中呈哑铃形生长的11例肿瘤中．有9例获得显微镜下全切除。结论术前MRI对高颈段椎管肿瘤有较好的诊断价值，而显微神经外科手术则有助于此类肿瘤的全切除。     

Intramedullary anaplastic oligodendroglioma in a child

Primary intramedullary anaplastic oligodendroglioma is a rare tumor, only four cases of which have been reported. The authors present the case of a 38-month-old boy with primary intramedullary anaplastic oligodendroglioma. He underwent partial removal of the tumor and spinal radiation therapy. The residual tumor disappeared 12 months after radiation, and 48 months after treatment there was no evidence of recurrence. This case shows that in primary intramedullary anaplastic oligodendroglioma, postoperative radiation therapy confined to the spinal cord can yield an optimal result.     

Symptomatic spinal cord metastasis from cerebral oligodendroglioma

Spinal subarachnoid spread is not uncommon in brain oligodendrogliomas; on the other hand, symptomatic involvement of the spinal cord and cauda is very rare, with only 16 reported cases. We report the case of a 41-year-old man who underwent resection of a low-grade frontal oligodendroglioma 4 years previously. He was again observed because of bilateral sciatic pain followed by left leg paresis. A spine MRI showed an intramedullary T12-L1 tumor with root enhancement. At operation, an intramedullary anaplastic oligodendroglioma with left exophytic component was found and partially resected. Two weeks later, a large left frontoparietal anaplastic oligodendroglioma was diagnosed and completely resected. The patient was neurologically stable for 8 months and died 1 year after the spinal surgery because of diffuse brain and spinal leptomeningeal spread. The review of the reported cases shows that spinal symptomatic metastases can occur in both low-grade and anaplastic oligodendrogliomas, even many years after surgery of the primary tumor; however, they exceptionally occur as first clinical manifestation or as anaplastic progression. The spinal seeding represents a negative event leading to a short survival.     

脊髓髓内肿瘤的显微手术治疗

目的介绍脊髓髓内肿瘤的显微手术治疗经验。方法显微手术治疗脊髓髓内肿瘤40例,随访术后神经功能状态,并对髓内肿瘤显微手术治疗的手术时机、手术技巧、脊柱稳定性及术后是否放疗等问题进行探讨。结果肿瘤全切除率75%,其中室管膜瘤全切除率94.8%,星形细胞瘤全切除率14%。出院时神经功能障碍加重14例,改善或稳定26例。结论及时采用显微外科技术切除肿瘤是提高脊髓髓内肿瘤病人生存质量的主要治疗手段。     

术中超声在脊髓髓内室管膜瘤和星形细胞瘤外科治疗中的应用

目的探讨术中超声在脊髓髓内室管膜瘤和星形细胞瘤显微外科手术中的应用价值。方法回顾性分析2010年1月至2018年5月华中科技大学同济医学院附属同济医院神经外科收治的78例脊髓髓内室管膜瘤和34例脊髓髓内星形细胞瘤患者的临床资料。根据术中是否使用超声辅助,分别将室管膜瘤和星形细胞瘤患者分为超声组(前者44例,后者18例)和对照组(前者34例,后者16例)。对所有患者行门诊或电话随访,通过影像学复查和改良McCormick量表(MMS)分级评估肿瘤复发和脊髓功能恢复情况。分别比较室管膜瘤和星形细胞瘤两组患者的疗效,并评价术中超声对肿瘤完全切除率的评估准确率。结果两组室管膜瘤和星形细胞瘤患者的性别、年龄、首诊症状、MMS分级及肿瘤累及脊髓节段的差异均无统计学意义(均P>0.05),基线资料均基本一致。室管膜瘤的超声组和对照组患者肿瘤完全切除率[分别为97.7%(43/44)、91.2%(31/34)]、术后并发症发生率[分别为8.8%(3/44)、11.8%(4/34)]及术后3个月脊髓功能恢复良好率[分别为36.4%(16/44)、32.4%(11/34)]的差异均无统计学意义(均P>0.05)。超声组和对照组的星形细胞瘤患者术后并发症发生比例(分别为:0/18、2/16)和术后3个月脊髓功能恢复良好比例(分别为:3/18、2/16)的差异均无统计学意义(均P>0.05);但与对照组比较,超声组的肿瘤完全切除比例高[分别为16/18、9/16,P<0.05]、无进展生存期(PFS)长[中位PFS分别为84.0(67.5~100.5)个月、75.0(52.0~98.0)个月,P<0.05]。以增强MRI为标准,术中超声判断室管膜瘤和星形细胞瘤全切除的准确比率分别为97.7%(42/43)、14/16。结论术中超声有助于实时、准确地判断脊髓髓内室管膜瘤和星形细胞瘤的肿瘤切除程度,且对星形细胞瘤的应用价值较大。     

Results of the operative treatment of intramedullary gliomas

BACKGROUND AND PURPOSE: To assess the results of the operative treatment of intramedullary gliomas. MATERIAL AND METHODS: In 1995-2002, 30 patients with intramedullary tumors were treated surgically in the Department of Neurosurgery in Sosnowiec. Among them 23 intramedullary gliomas were recognized. There were 8 females and 15 males aged from 11 to 61. The condition of the patients before the operation was assessed according to a modified McCormick's scale. Only two patients were found to be in a good condition -- I degree, 17 patients (74%) -- II degree or III degree and 4 patients were in a serious condition -- IV degree. Among the operated tumors only 9 had a clear border. There were 5 ependymomas (grade I) and 4 anaplastic ependymomas (grade III). The remaining ones were astrocytomas of varying malignancy and infiltration of the white matter of the spinal cord: 8 cases of fibrillary astrocytoma, 3 cases of gemistocytic astrocytoma, but one anaplastic and one case of pilocytic astrocytoma, oligoastrocytoma mixtum and anaplastic oligoastrocytoma. RESULTS: Control MRI revealed a total removal of the lesion in 16 patients (70%), gross total removal in 5 patients (21%), partial removal in two patients (8%). Four patients died among those operated on: two in the perioperative period, the other two 9 months and 2 years after the surgery, respectively. In four patients there was observed regrowth of the lesion and one patient was re-operated on. In the second case fatal recurrence in region C2-Th6 was observed and the patient died, in the last two cases the patients were stable and thus it was decided to delay the re-operation. CONCLUSION: Low mortality, morbidity and recurrence rates recommend surgery as an effective treatment for intramedullary gliomas.     

Apoptosis in glial tumors as determined by in situ nonradioactive labeling of DNA breaks

Tumor growth depends on cell division and cell death. To investigate the role of apoptosis in tumor cell death, we examined 83 cases of glial tumors using in situ nonradioactive tailing of DNA breaks. In addition, since p53 protein may participate in the regulation of apoptosis in glioblastoma, we compared the apoptosis ratio (AR) with the labeling index (LI) of p53 protein immunopositivity. The AR in glial tumor parenchyma ranged from 0 to 1.4%: mean AR ± standard deviation was 0.4 ± 0.4% (range, 0–1.4) for glioblastoma, 0.3 ± 0.3% (range, 0.01– 0.83) for anaplastic astrocytoma, 0.1 ± 0.1% (range, 0– 0.41) for low-grade astrocytoma, 0.006 ± 0.008% (range, 0–0.02) for pilocytic astrocytoma, 0.2 ± 0.2% (range, 0– 0.62) for oligodendroglioma and 0.003 ± 0.004% (range, 0–0.01) for ependymoma. ARs were significantly higher in higher-grade astrocytic tumors than in lower-grade tumors (Mann-Whitney U test: P = 0.0003), although wide variability in each group resulted in overlapping between the groups. p53 protein immunopositivity (more than 25% of nuclei) was found in 15 of 32 glioblastoma cases, while in the remaining 17 none or only a low percentage (up to 6%) of the nuclei were positive. In p53 protein-positive cases mean AR (0.51 ± 0.47%) was not significantly higher than that in p53 protein-negative cases (0.22 ± 0.23%; P = 0.1681). Received: 17 February 1995 / Revised: 12 June 1995 / Accepted: 30 August 1995     

Oligodendroglioma of the “widespread” type in the spinal cord

The authors present clinical, radiological, and pathological features of the “widespread” type of primary spinal oligodendroglioma arising in a 12-year-old boy. The initial symptoms were gait disturbance, hypesthesia of the lower trunk, and scoliosis. Gadolinium-enhanced magnetic resonance imaging revealed an intramedullary tumor with syringomyelia. The tumor spread rapidly in a “widespread” pattern within the spinal cord between C7 and T12. One year after surgery the patient had made a poor neurological recovery despite the two-stage tumor resection. Only five cases of widespread tumors, including our case, have been described with discussion of their clinical manifestations. Received: 14 July 1997 Revised: 27 February 1998     

神经电生理监测和超声技术在脊髓髓内肿瘤显微手术中的应用

目的探讨神经电生理监测及超声技术在脊髓髓内肿瘤显微切除术中的应用价值。方法回顾性分析10例脊髓髓内肿瘤病人的临床资料。均行肿瘤显微切除术,术中以体感诱发电位、自发肌电图监测和超声定位辅助肿瘤切除。结果肿瘤全切除8例,大部分切除2例。术后病理诊断:室管膜瘤6例,星形细胞瘤1例,少枝胶质细胞瘤1例,神经鞘瘤1例,蛛网膜囊肿1例。出院时神经功能改善或稳定9例,加重1例;无死亡病例。结论显微手术是治疗脊髓髓内肿瘤的有效措施,术中辅助神经电生理监测及超声技术可提高肿瘤切除率,最大程度保护神经功能,改善病人预后。     

Intracranial dissemination of primary spinal cord anaplastic oligodendroglioma

We report the first case of a 22-year-old man, with a previously neurosurgically treated intramedullary anaplastic oligodendroglioma (World Health Organization grade III), who developed 19 months later two histologically proven intracranial metastases. We support a hypothesis whereby the anaplastic parts of tumors have spread along the spinal cord and brainstem via the cerebrospinal fluid pathways, a process that could be promoted by surgical manipulation, although the relative contribution of the two factors remains speculative.     

Intramedullary mass lesions of the spinal cord

Intramedullary tumors form 10% of all the spinal cord tumors. Majority of them are gliomas and rarely benign tumors are encountered. Twenty five cases of intramedullary mass lesions are studied regarding their clinical presentation, radiological features, surgical aspects and outcome following the surgery and radiotherapy. There were six benign lesions where the outcome was excellent. There were 11 patients with astrocytoma and 8 cases with ependymoma. In all, arrest of the disease or improvement could be achieved in 20 cases. Extent of surgical resection and role of radiotherapy, which are still controversial, are discussed.     

Spinal deformity after resection of cervical intramedullary spinal cord tumors in children

Objective Progressive spinal deformity after cervical intramedullary spinal cord tumor (IMSCT) resection requiring subsequent fusion occurs in many cases among pediatric patients. It remains unknown which subgroups of patients represent the greatest risk for progressive spinal deformity. Materials and methods The data for 58 patients undergoing surgical resection of cervical IMSCT at a single institution were retrospectively collected and analyzed for development of progressive spinal deformity requiring fusion. The association of all clinical, radiographic, and operative variables to subsequent progressive spinal deformity as a function of time was assessed via Kaplan–Meier plots and Log-rank and Cox analyses. Results Mean age at the time of surgery was 11 ± 6years. Eleven (19%) patients required subsequent fusion for progressive spinal deformity at a median [interquartile range (IQR)] of 4 (2–6) years after IMSCT resection. Five (36%) of 14 patients with preoperative scoliosis or loss of lordosis developed postoperative progressive spinal deformity compared to only 6 (13%) of 44 patients with normal preoperative sagittal and coronal balance, p = 0.06. Patients <13years of age were more than three times more likely to develop postoperative progressive deformity, p = 0.05. Decompression spanning both the axial cervical spine (C1–C2) and the cervico–thoracic junction (C7–T1) increased the risk for progressive spinal deformity fourfold, p = 0.04. Number of spinal levels decompressed, revision surgery, radiotherapy, involvement of C1–C2 or C7–T1 alone in the decompression, or any other recorded variables were not associated with progressive postoperative spinal deformity. Conclusion Patients possessing one or more of these characteristics should be monitored closely for progressive spinal deformity after surgery.     

Long-term survival of glioblastoma multiforme: importance of histopathological reevaluation

The overall prognosis for patients with glioblastoma multiforme is extremely poor. However, a small proportion of patients enjoy prolonged survival. This study investigated retrospectively the extent to which erroneous histopathological classification may contribute to long-term survival of patients initially diagnosed with “glioblastoma multiforme”. We compared two age- and gender-matched patient groups with different postoperative time to tumor progression (TTP), defined as “short-term” for TTP of less than 6 months (n=54) and “long-term” for TTP of more than 12 months (n=52). Histological specimens of the corresponding tumors, all primarily diagnosed as glioblastome multiforme, were reevaluated according to the current World Health Organization (WHO) classification of central nervous system tumors, with the investigators being blinded to clinical outcome. Among the tumors from short-term TTP patients, one tumor (2%) was reclassified as anaplastic oligoastrocytoma (WHO grade III) while the remaining 53 were confirmed as glioblastoma multiforme. In contrast, 13 tumors (25%) from the long-term TTP patients were reclassified, mostly as anaplastic oligodendroglioma (WHO grade III; n=7) or anaplastic oligoastrocytoma (WHO grade III, n=2), respectively. In addition, three were reclassified as anaplastic astrocytoma (WHO grade III), and one was identified as anaplastic pilocytic astrocytoma (WHO grade III). Our data indicate that a sizable proportion of glioblastoma patients with long-term survival actually carry malignant gliomas with oligodendroglial features. The correct histopathological recognition of these tumors has not only progrostic but also therapeutic implications, since oligodendroglial tumors are more likely to respond favorably to chemotherapy. Received: 9 November 1999, Received in revised form: 13 January 2000, Accepted: 3 February 2000     

Surgical management of spinal intramedullary tumors: Ten-year experience in a single institution

Despite their rare occurrence, intramedullary spinal cord tumors can cause considerable morbidity and mortality without treatment. Timing of surgery, extent of resection and selection of favorable treatment option are important considerations for a good surgical outcome. In this clinical study, we report our patient series and convey our treatment strategy. We retrospectively reviewed 91 patients with primary intramedullary spinal cord tumors who underwent microsurgical resection at our institution between 2008 and 2018. Data were collected consisting of age, sex, location and histology of tumor, extent of resection, presenting symptoms and neurological outcomes. Modified McCormick Scale was used to assess neurological status of patients. 47 female and 44 male patients were followed-up for a mean period of 35.7 months. The most frequent pathological diagnosis was ependymoma in 56 patients, followed by astrocytoma in 21 and hemangioblastoma in 5 patients. The rest of the tumors consisted of 3 cavernomas, 3 mature cystic teratomas, 2 PNET, one epidermoid tumor. Gross total resection was achieved in 67 patients, while subtotal resection and biopsy was performed in 15 and 9 respectively. The most commonly involved localization was cervical (n = 39), followed by thoracic region (n = 24). Despite immediate postoperative worsening of neurological status, a great number of patients improved at the last follow-up. Gross total resection remains the primary goal of treatment while adjuvant radiation and/or chemotherapy may be alternative options for high grade tumors. Preoperative neurological status was the most important and the strongest predictor of functional outcome.     

Pineal tumors: experience with 48 cases over 10 years

The authors retrospectively reviewed 48 patients treated at Seoul National University Hospital (SNUH) between 1986 and 1995. There were 35 children and 13 adults, accounting for 10.1% of 345 pediatric and 0.68% of 1914 adult brain tumors in SNUH during the same period. The 48 cases consisted of 33 cases of germ cell tumor (69%, GCT); 6 of pineoblastoma (PB, 12.5%); 3 of pineocytoma (PC, 6.3%); 3 of anaplastic astrocytoma (6.3%); 1 of astrocytoma; 1 of glioblastoma; and 1 of ependymoma. The median age was 13 years (range 1–59) and the male-to-female ratio was 3.36:1. The most frequent presenting symptom was due to increased intracranial pressure (90%), followed by Parinaud syndrome or diplopia (50%). Patients with a benign tumor, such as teratoma (TE), astrocytoma, or ependymoma, underwent surgery by the occipital transtentorial approach (OTT) for attempted radical resection without adjuvant therapy, while patients with immature teratoma (imTE), PC, and anaplastic astrocytoma underwent regional radiotherapy (RT) after debulking via OTT. Seven patients with nongerminomatous malignant GCT (NG-MGCT) and 3 with germinoma (GE) underwent craniospinal radiation only, 6 with GE, a NG-MGCT, and 2 with GE+TE received craniospinal radiotherapy (CSRT) after debulking via OTT. Three patients with GE, 4 with NG-MGCT, and 3 with PB underwent radiochemotherapy after debulking via OTT. Forty-four patients were followed up after treatment. The median follow-up period was 36 months. All patients with GE were alive after RT at 36 months (median) of follow-up (range 7–70 months). All with GE+TE and TE were alive. Three patients with PC or astrocytoma were also alive with stable or no evidence of disease. In 1 of the 3 cases of imTE there was a recurrence. However, 4 patients with NG-MGCT died, all of whom had undergone CSRT only; 2 PB patients were alive (12, 19 months), 1 in a moribund status (36 months), and 2 were dead (6, 60 months). The overall mean survival time with pineal tumors was 66 months and the 3-year survival rate was 84% with minimal posttreatment complications. It is concluded that pineal region tumors have male and childhood predominances, and the most common tumor is GCT. The majority of pineal region tumors are malignant. Pineal region tumors can be approached safely and effectively and the surgical complications are mostly transient. Their prognosis is dependent on the pathologies and treatment modalities.     

脊髓髓内肿瘤显微外科治疗

目的 总结并探讨脊髓髓内肿瘤显微外科手术的治疗经验。方法 统计近3年来显微外科手术治疗18例髓内肿瘤的临床资料、手术方式及术后转归。结果 手术全切17例,大部分切除1例;病理学诊断室管膜瘤8例,星形细胞瘤6例,血管母细胞瘤3例,血管畸形1例;术后运动及感觉障碍改善14例,不明显3例,加重1例;随访共16例,未见1例复发。结论 髓内肿瘤一旦诊断应早期手术治疗。脊髓髓内肿瘤应强调显微手术镜下操作,这不仅使病变与正常脊髓更易辨清,同时使操作更为细致轻柔。仔细辨认肿瘤与脊髓界限,对全切肿瘤和保护脊髓是有帮助的。对于肿瘤与脊髓有较清楚界限时应争取显微镜下全切,但当肿瘤侵蚀已至软膜下区时,此时不能盲目追求全切,以避免永久性功能障碍。     

脊髓室管膜瘤的显微外科治疗

目的探讨脊髓室管膜瘤显微外科治疗中的相关问题。方法回顾性分析22例脊髓室管膜瘤患者的临床资料。结果22例脊髓室管膜瘤中，肿瘤主体位于颈段12例，胸段7例，腰段3例。其中15例在肿瘤头端和，或尾端合并有脊髓空洞，囊性空洞呈分隔状5例，2例瘤体内有囊变。肿瘤全切15例，次全切除7例。根据治疗前后神经功能综合评价，好转14例，稳定7例，加重1例。结论对脊髓室管膜瘤应行积极的显微外科手术治疗，肿瘤能否全切与瘤体大小无明显关系，主要取决于肿瘤与脊髓的粘连程度；手术不能全切者术后辅以局部放疗可获得良好的疗效。     

弥散张量磁共振成像在脑实质内肿瘤手术中的作用

目的研究弥散张量磁共振成像(DTI)在认识脑实质内肿瘤的生长方式和显微手术中的作用。方法脑实质内肿瘤患者41例,随机分为对照组23例,研究组18例。手术前、后行常规MR及DTI检查。所有患者手术均在显微操作下进行。两组病例依据不同的影像选择手术入路。比较两组患者手术结果、DTI影像变化以及手术前后神经功能改变。结果21例胶质母细胞瘤和1例室管膜母细胞瘤位于中央区及附近、颢枕部和颞叶深部,术前DTI表现肿瘤仅将传导束推移；2例转移瘤位于颞枕部,术前DTI显示视放射没有解剖移位,而是部分缺如,提示肿瘤对其有侵犯；8例星形或少枝胶质瘤位于额、颞、顶、枕部,术前DTI提示传导束未出现移位、变形,表现为经过肿瘤部位的传导束中断,这说明肿瘤对其侵犯、破坏；其余肿瘤未涉及到重要传导束。术后DTI显示对照组5例患者因手术导致传导束受损,研究组2例损伤。传导束损伤的患者均出现相应的神经功能障碍。结论DTI能够清楚可靠的显示肿瘤与白质传导束的关系,有利于确定手术方案,术中保护功能传导束,提高肿瘤切除率。