基于美国SEER数据库的儿童胶质瘤临床特点和预后分析

目的基于美国SEER数据库回顾总结儿童胶质瘤的临床特点,并探究不同组织学类型的生存预后相关影响因素。方法纳入SEER数据库中2000-2015年经组织病理学证实的胶质瘤患儿共7759例,根据组织学类型分为毛细胞型星形胶质瘤、髓母细胞瘤、室管膜瘤、胶质母细胞瘤及其他类型胶质瘤,绘制Kaplan?Meier生存曲线,比较毛细胞型星形细胞瘤、髓母细胞瘤、室管膜瘤和胶质母细胞瘤患儿的5年生存率;采用单因素log?rank检验和多因素向前引入法Cox回归分析筛查上述4种组织学类型患儿生存预后相关影响因素。结果本组7759例患儿根据组织学类型分为毛细胞型星形细胞瘤2585例(33.32%)、髓母细胞瘤2061例(26.56%)、室管膜瘤777例(10.01%)、胶质母细胞瘤443例(5.71%)、其他类型胶质瘤1893例(24.40%)。不同组织学类型之间性别(χ²=60.390,P=0.000)、年龄分布(χ²=600.318,P=0.000)、肿瘤大小(χ²=90.773,P=0.000)和原发部位(χ²=2117.948,P=0.000)、病理分级(χ²=1233.506,P=0.000)差异均有统计学意义,其中,髓母细胞瘤和胶质母细胞瘤好发于男性[60.89%(1255/2061)和57.56%(255/443)];学龄期为高峰发病年龄[35.48%(2753/7759)];毛细胞型星形细胞瘤和髓母细胞瘤以小肿瘤为主[31.30%(809/2585)和33.77%(696/2061)]且好发于小脑[42.24%(1092/2585)和59.58%(1228/2061)],室管膜瘤和胶质母细胞瘤则较大[32.43%(252/777)和29.57%(131/443)]且多发生于幕上及脑室[36.81%(286/777)和65.91%(292/443)],仅毛细胞型星形细胞瘤以低级别为主[24.06%(622/2585)]。生存分析显示,毛细胞型星形细胞瘤、髓母细胞瘤、室管膜瘤和胶质母细胞瘤患儿5年生存率分别为97.41%(2518/2585)、64.39%(1327/2061)、76.83%(597/777)和16.25%(72/443),不同组织学类型之间差异有统计学意义(χ²=2145.672,P=0.000)。单因素log?rank检验和多因素Cox回归分析显示,不同组织学类型胶质瘤预后相关影响因素不尽一致,主要包括确诊年龄、肿瘤大小和原发部位、病理分级、手术切除和放疗,除毛细胞型星形细胞瘤外,髓母细胞瘤、室管膜瘤和胶质母细胞瘤患儿接受手术切除均对生存预后有积极意义;放疗可降低髓母细胞瘤患儿死亡风险,但可能增加毛细胞型星形细胞瘤患儿的死亡风险。结论毛细胞型星形细胞瘤比例最高,预后较好;胶质母细胞瘤发病率相对较低,预后较差。人口统计学信息、肿瘤特征和治疗方案均为儿童胶质瘤患者生存预后的预测因素,手术切除可降低除毛细胞型星形细胞瘤外的其他组织学类型胶质瘤患儿的死亡风险;放疗可降低髓母细胞瘤患儿的死亡风险,但有可能增加毛细胞型星形细胞瘤患儿的死亡风险。     

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.     

儿童室管膜瘤的治疗进展与预后

室管膜瘤起源于脑室系统室管膜细胞及其下的胶质上皮细胞,占颅内肿瘤1.2%~3.5%,占成人脑肿瘤的不到3%,但占儿童颅内肿瘤的8%~10%[1]。室管膜瘤在所有儿童中枢神经系统肿瘤的发病率中位居第三。目前在美国每百万儿童中室管膜瘤的发生人数是2.2人,而低分级星形细胞瘤和髓母细胞瘤的发生率分别是每百万分之7.5和5.7[2]。室管膜瘤在儿童中的发病率高于成人,在16~64岁年龄组为1.5人/百万人口,在65岁以上年龄组为1.6人/百万人口。69%的颅内室管膜瘤(48例中的33例)发生在儿童,96%的髓内室管膜瘤(53例中的51例)发生在成人。从年龄分布上看,66%的儿童…     

脊髓髓内亚室管膜瘤

亚室管膜瘤(Subependymoma)是缓慢生长的肿瘤,大多数髓内肿瘤是室管膜瘤和星形细胞瘤,唯一区分这些肿瘤的方法仍然是病理学检查,病史,体格检查和放射影象学检查甚至术中肉眼观察都不能区分这三种肿瘤。本文报导一组6例脊髓髓内亚室管膜瘤病例。 该6例为1981.1～1993.8接受手术切除脊髓髓内亚室管瘤的病人。肿瘤除1例位于脊髓胸腰段外,均位于颈髓或脊髓颈胸段,每例均行肉眼全切,无一例术后接受放射治疗,3例入院前进行过手术活检和放疗。     

延颈髓内巨大髓内肿瘤二例报告并文献复习

髓内肿瘤比较少见,在神经系统肿瘤中大约占4%～10%;在髓内肿瘤中室管膜瘤和星形细胞瘤比较常见,占所有髓内肿瘤的70%[1].我院在2007年6月至7月手术切除1例延髓至颈胸段长节段髓内室管膜瘤和1例胶质母细胞瘤,现报告如下.     

儿童第四脑室肿瘤的显微外科治疗(附43例报告)

目的 探讨儿童第四脑室常见肿瘤的诊断和显微手术技巧.方法 回顾性分析2006~2010年手术治疗的43例儿童第四脑室肿瘤患者的临床表现与影像学等临床资料;根据肿瘤与小脑蚓部和第四脑室的关系,设计个体化手术入路切除肿瘤.结果 肿瘤全切除38例,次全切5例(为与第四脑室底粘连紧密者).绝大多数病例无明显并发症或并发症较轻,经治疗后好转.病理结果示髓母细胞瘤27例,室管膜瘤8例,星形细胞瘤7例(其中毛细胞型3例),神经细胞瘤(交界性)1例.髓母细胞及WHOⅢ级的室管膜瘤术后进行了全脑和脊髓放疗,次全切的室管膜瘤和星形细胞瘤进行了局部放疗.结论 儿童第四脑室肿瘤以髓母细胞瘤最多见,其次是室管膜瘤和星形细胞瘤;正确的入路选择和手术技巧是取得良好疗效、减少并发症的关键.     

儿童脊髓肿瘤手术和放疗并用的治疗效果

报道1959～1990年31例(男17例,女14例)<17岁儿童脊髓原发性肿瘤手术与放射联合治疗的结果。28例有组织学诊断者15例为星形细胞瘤,11例为室管膜瘤,混合性少突星形细胞瘤及神经节胶质瘤各1例,3例病史不明。星形细胞瘤中15例中12例,室管膜瘤11例中8例为低恶性,前者年龄0～16岁(中数5),后者8～16岁(中数12)。颈髓5例,颈胸9例,胸3例,胸腰6例,腰8例。室管膜瘤多位于远端,11例中7例位于L1～5,累及马尾、终丝或脊髓圆锥。15例星形细胞瘤中无1例位于远端。所有患者在作了诊断性脊髓造影后,继而作了椎板切除减压,31例中11例(35％)未切除肿瘤,14例     

显微手术治疗脊髓髓内胶质瘤23例

目的:分析23例脊髓髓内胶质瘤显微手术治疗经验,评价其疗效和预后。方法:对9年来经显微手术治疗的23例脊髓髓内胶质瘤患者的肿瘤病理类型、肿瘤部位、临床症状、体征、辅助检查和临床疗效进行回顾性分析研究。结果:本组13例星形细胞瘤(56.5%)及10例室管膜瘤(43.5%),均于显微镜下肉眼全切除,全组无围手术期死亡。16例随访2个月至7.6年[平均(38±11)个月]。末次随访结束时(失访者按出院前的神经功能评定):肌力改善16例(69.6%)、不变2例(8.7%)、加重2例(8.7%)及死亡3例(13.0%)。随访中复发5例。结论:脊髓髓内胶质瘤宜早诊断并尽早接受手术治疗。显微神经外科技术可提高肿瘤全切,改善脊髓髓内胶质瘤的预后。星形细胞瘤应及时进行术后放疗和化疗。     

采用一侧椎板切除术摘除脊髓肿瘤

作者从1983年起对19例脊髓肿瘤患者(男8例,女11例,年龄16～62岁)施行20次手术,1例因术后1年半复发而再手术,采用一侧椎板切除通路实现肿瘤摘除。硬脊膜外肿瘤6例(转移瘤3例、网状细胞肉瘤2例、浆细胞瘤1例)。髓外肿瘤10例(神经纤维瘤4例、脊膜瘤5例、室管膜瘤1例),髓内肿瘤3例(星形细胞瘤2例、血管瘤1例)。10例髓外肿瘤中位于脊髓前侧面有4例,后侧面3例,侧面2例,后面1例。一侧椎板切除,切除1个椎弓有2例,2个椎弓9例,3个椎弓4例,4个椎弓2例,6个椎弓2例。10例完全摘除肿瘤(全部为神经纤维瘤、脊膜瘤、室管膜瘤),9例只作肿瘤部     

小儿幕上胶质瘤形态学和病理原因的研究

儿童脑肿瘤主要影响到旧的大脑结构、脑干、小脑和脊髓。它们常以髓母细胞瘤、室管膜瘤、星形细胞瘤(如少枝胶质母细胞瘤)出现,相反,新脑的神经外胚叶肿瘤不常见,成人肿瘤的特点(如     

Surgical experience with resection en bloc of intramedullary astrocytomas and ependymomas in the cervical and cervicothoracic region.

We performed 8 operations on 7 patients with benign intramedullary astrocytomas and ependymomas in the cervical and cervicothoracic region. All patients initially underwent gross total tumor resection en bloc. One patient with an astrocytoma showed tumor recurrence postoperatively, and underwent a second operation resulting in subtotal removal. The follow-up after the initial surgery ranged from 2.7 to 19.7 years (mean 8.5 years). Symptomatic improvement was observed in 6 patients after the initial operation. Two patients showed postoperative neurological deterioration, one with an ependymoma and the other after the second operation. No operative complications or deaths, nor postoperative respiratory dysfunction occurred. Benign intramedullary astrocytomas and ependymomas of the cervical and cervicothoracic spinal cord can be treated by radical resection en bloc with a low morbidity and recurrence, as well as acceptable outcomes. We describe here the surgical technique for en bloc tumor removal.     

Thoracic exophytic ependymoma masquerading as a benign extra-axial tumor

Spinal tumors are conventionally differentiated based on location in relation to the spinal cord. Benign spinal tumors such as schwannomas and meningiomas are typically extra-axial (intradural extramedullary) lesions, whereas more aggressive primary spinal tumors such as ependymomas are typically intramedullary masses. Rarely, ependymomas can have both intramedullary and extramedullary components (typically referred to as exophytic ependymomas). We report a case of a spinal exophytic ependymoma that radiographically masqueraded as a benign intradural extramedullary lesion causing cord compression and neurologic deficit in a 47-year-old man. The diagnosis of exophytic ependymoma was made intra-operatively, with resultant gross total resection of the extramedullary portion and subtotal resection of the intramedullary portion. Histopathological examination confirmed ependymoma with World Health Organization grade II/IV. Pre-operative suspicion of an exophytic ependymoma influences operative planning and clinical management. We review the literature and discuss clinical management strategies for these interesting spinal tumors.     

Sensitivity and specificity of epithelial membrane antigen staining patterns in ependymomas

Pattern and extent of epithelial membrane antigen (EMA) immunoreactivity in ependymomas as compared to other glial tumors have only been investigated in small series. To determine sensitivity and specificity of EMA staining, 54 ependymomas were evaluated in comparison to 54 glioblastomas, 43 fibrillary astrocytomas and 21 oligodendrogliomas. Distinct punctate intracytoplasmic EMA immunoreactivity was observed in 48/54 ependymomas (89%), whereas ring-like EMA staining was observed in 17/54 ependymomas (31%). Apart from the absence in most myxopapillary ependymomas, neither staining pattern was related to tumor grade or localization. Dot-like EMA immunoreactivity was less frequently observed in glioblastomas [32/54 (59%), P<0.05 vs ependymomas], fibrillary astrocytomas [10/43 (23%), P<0.001 vs ependymomas] and oligodendrogliomas [2/21 (10%), P<0.001 vs ependymomas], whereas ring-like EMA staining was absent. Sensitivity and specificity of punctate EMA staining for the diagnosis of ependymoma as compared to other glial tumors were determined: A finding of 5 EMA dots/high-power field was associated with a sensitivity of 72% and a specificity of 81%. The presence of ring-like EMA positive structures was less sensitive (32%), but highly specific (100%). To conclude, distinct punctate and ring-like EMA staining might serve as sensitive and specific markers of ependymal differentiation in glial tumors and, thus, may aid the diagnosis of ependymoma.     

Cortical ependymoma or monomorphous angiocentric glioma?

Ependymoma is the third most common childhood intracranial tumor after medulloblastoma and pilocytic astrocytoma. Most ependymomas occur in the posterior fossa and spinal cord but only five cases confined to the cerebral cortex have been reported. The current case is a 5‐year‐old boy with a somewhat ill‐defined cortical tumor diagnosed as pilocytic astrocytoma on biopsy, and treated with radiotherapy. Nine years later, resection of the essentially unaltered tumor was performed for treatment of intractable seizures. Histologically, the tumor had some areas with the typical appearance of ependymoma as well other areas which contained piloid cells. There was also evidence of focal infiltrative growth. These findings bore resemblance to a recently described entity monomorphous angiocentric glioma/angiocentric neuroepithelial tumor, which combines features of ependymoma with pilocytic and diffuse astrocytomas. Both cortical ependymomas and angiocentric monomorphous glioma/angiocentric neuroepithelial tumor appear to be low‐grade tumors although their rarity makes accurate prognosis problematic. The current case has features of both entities, suggesting they may be closely related.     

Intramedullary astrocytomas and ependymonas in the pediatric age group: a retrospective study

A series of 45 pediatric patients underwent surgery for intramedullary astrocytoma or ependymoma at the Cook County Hospital or the Children's Memorial Hospital of Chicago (Northwestern University) and the Neurosurgical Department of Rome La Sapienza University. Results showed that intramedullary astrocytomas and ependymomas in children differ from one another in terms of prognosis, and particularly as regards surgical strategy as an element of therapeutic management. Gross total removal of 70% of the ependymonas was achieved, in comparison to 33% of the astrocytomas. In astrocytomas the extent of resection did not significantly influence prognosis. Neurological condition on admission, regardless of oncotype, was found to be extremely influential: patients operated in good condition tended to remain neurologically stable or improve in the long term, while those operated on while in poor condition did not show any improvement at all. No definitive conclusions were possible regarding the value of radiotherapy, because it was only performed in 6 of the 45 cases. The authors limited its use to highly malignant lesions in view of the well-known sensitivity of the spinal cord to radiation, especially in children.     

Spinale intramedulläre Tumoren

OBJECTIVE: Timing of surgery in patients with intramedullary tumors is the subject of controversy. The aim of this retrospective study is to evaluate whether patients with intramedullary ependymomas and astrocytomas have a better postoperative prognosis without or with slight preoperative deficits than those with severe preoperative neurological disturbances. PATIENTS AND METHODS: During a period of 8.5 years (January 1992-August 2000), 34 patients with intramedullary tumors underwent surgery in our Neurosurgical Department. Among them there were five astrocytomas WHO grade II and ten ependymomas WHO grade II. Recurrences were observed in two patients with astrocytomas and one patient with ependymoma. Pre- and postoperative functional performance was classified according to the McCormick scale in grade I-IV (grade I: neurologically normal or mild focal deficit and grade IV: severe neurological deficits and without functional independence). The follow-up period varied from 4 to 76 months (mean: 27.9 months). RESULTS: All seven grade I patients remained unchanged after surgery. Two of four grade II patients improved to grade I; two deteriorated to grade III. Two of three grade III and IV patients remained unchanged and another one deteriorated from grade III to IV. CONCLUSION: At the best, intramedullary astrocytomas and ependymomas should be operated when symptoms are mild. Early surgery can achieve good functional outcome.     

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.     

多节段脊髓室管膜瘤的显微外科治疗

目的 探讨多节段脊髓室管膜瘤的手术技术和手术治疗疗效。方法 本文回顾分析了2003年至2005年治疗的10例多节段脊髓室管膜瘤的临床资料。结果 颈髓室管膜瘤6例，胸髓室管膜瘤3例，1例术后复发肿瘤累及胸、腰髓并累及椎旁。平均累及脊髓9．4个节段。双下肢截瘫4例，高位截瘫6例，括约肌功能障碍5例，呼吸困难4例。MRI可见脊髓空洞形成8例。手术采用椎板复位9例，C-D钉棒系统脊柱后路内固定1例。术后患者肌力恢复7例，无改变3例。呼吸困难，应用呼吸机辅助呼吸1例。术后复查MRI无肿瘤残留9例，1例部分椎旁肿瘤残留。结论 采用显微神经外科技术可以达到镜下多节段室管膜瘤全切，患者术后症状大多数可以明显改善。多节段椎板切开术后应考虑椎板复位或脊柱内固定，以保持脊柱的稳定性。     

电生理监测下高颈段髓内室管膜瘤的外科治疗

目的 探讨高颈段髓内室管膜瘤的显微外科治疗.方法 回顾性分析23例高颈段脊髓髓内室管膜瘤,均行显微外科切除,术中均采用联合体感诱发电位(SEP)和运动诱发电位(MEP)监测辅助肿瘤切除.结果 手术全切除肿瘤17例,近全切或大部分切除5例,部分切除1例;全组无手术死亡;术后门诊或电话随访22例,随访3个月至6年,神经功能改善16例(73%),稳定无变化4例(18%),加重2例(9%),随访期间无复发;术中诱发电位结果:真阴性18例(18/19),假阴性1例,真阳性2例(2/3).结论 尽早显微手术切除是高颈段脊髓髓内室管膜瘤的有效治疗措施,术中联合SEP和MEP监测可提高肿瘤全切率,减少术后并发症,最大程度地稳定和改善神经功能.

Abstract：

Objective To investigate the mieroneurosurgcial management for intmmedullary spinal ependymomas in the superior cervical spinal cord.MethodsClinical data of 23 patients with intramedullary ependymoma in the superior cervical spinal cord were analyzed retrospectively.All the patients were treated by microsurgery assisted with somatosensory evoked potential(SEP)and motor evoked potential(MEP).Results Total removal was achieved in 17 cases,subtotal removal in 5 cases and partial resection in 1 case.No patients died.A follow up of 3 months to 6 years by out-patient review and telephone interview were performed in 22 cases:neurological function was improved in 16 cases(73%),no changes occurred in 4(18%),deteriorated in 2(9%)and none was recurrence.Results of introperative monitoring:18 patients presented true negative findings,while 2 patient presented true positive findings,1 false positive findings.Conclusions Early microsurgical treatment is the most effective measure for intramedullary ependymoma in the superior cervical spinal cord.The combined applications of intraoperative SEP and MEP monitoring can increase removal rate,reduce postoperative complication,and improve the prognosis of the patients.