Ovarian and omental ependymomas in peritoneal washings: cytologic and immunocytochemical features

The cytologic findings in peritoneal washings of two women, one of whom had an ovarian ependymoma and the other a primary omental ependymoma, are reported. The ependymomas were characterized by the presence of numerous, isolated, spindle and stellate cells as well as groups of cells forming true rosettes. The tumor cells displayed slightly pleomorphic, round-to-oval eccentric nuclei and abundant fibrillary cytoplasm with tapering cytoplasmic processes. In addition, one of the patients had numerous papillae and cell clusters with associated psammoma bodies indistinguishable from those found in low-grade serous carcinoma. The demonstration of glial fibrillary acidic protein (GFAP) in both cases by immunocytochemical procedures indicates the usefulness of this method in cytologic preparations to confirm the diagnosis of these uncommon neoplasms.     

Clivus ependymoma

Summary An 8-month-old girl had an ependymoma in the clivus, 2x6 cm in size, connected with the fourth ventricle by a cord of tissue 0.5 cm thick. There were no indications to make us suspect the origin of the tumour in the fourth ventricle, or that it was a case of ependymoma.     

Intracranial ependymomas: Prognostic aspects

According to the grading of brain tumors as proposed by the WHO in 1976, out of 128 ependymomas 83 tumors could be classified as grade II and 38 as grade III Only seven subependymomas were benign and could be assigned to grade I.In contrast to most series known from the literature, 73 ependymomas were located above the tentorium and only 55 in the posterior cranial fossa. The grade of malignancy rised with an increased distance from the ventricular level.Macroscopically complete exstirpations were usually possible in hemispheric ependymomas, whereas tumors arising from the floor of the fourth ventricle often allowed only a partial removal. The operative mortality in the infratentorial group was more than twice as that in the supratentorial group.Postoperative survival was predominantly dependent on the histologic grade of malignancy. The five year survival rate without recurrence was 57.4% in grade II ependymomas as compared to 24.1% in grade III ependymomas. It could be improved by postoperative radiation therapy in both groups of malignancy. The almost identical longterm results indicate that even in less malignant ependymomas new tumor growth will occur later on.     

Treatment and survival of supratentorial and posterior fossa ependymomas in adults

Ependymoma is a rare primary brain or spinal cord tumor that arises from the ependyma, a tissue of the central nervous system. This study analyzed a large cohort of adult supratentorial and posterior fossa ependymoma tumors in order to elucidate factors associated with overall survival. We utilized the USA National Cancer Database to study adult World Health Organization grade II/III supratentorial and posterior fossa ependymoma patients treated between 1998 and 2011. Overall survival was estimated by the Kaplan–Meier method and factors associated with survival were determined using a multivariate Cox proportional hazards model. Among 1318 patients, 1055 (80.0%) had grade II and 263 (20.0%) anaplastic tumors located in the posterior fossa (64.3%) and supratentorial region (35.7%). Overall average age was 44.3 years, 48.0% of patients were female, 86.5% were Caucasian, and 36.8% underwent near/gross total surgical resection. Radiotherapy was given to 662 patients (50.8%) and 75 (5.9%) received chemotherapy. Older age at diagnosis (hazard ratio [HR] 1.51, p < 0.0001), high tumor grade (HR 1.82, p = 0.005), and large tumor size (HR 1.66, p = 0.008) were associated with poor survival. Females compared to males (HR 0.67, p = 0.03) and patients with posterior fossa tumors versus supratentorial (HR 0.64, p = 0.04) had a survival advantage. Our study showed that older patients, with supratentorial tumors, and high histological grade had an increased risk of mortality. A survival benefit was captured in females and patients with posterior fossa tumors. Adjuvant radiotherapy and chemotherapy did not confer a survival benefit among all patients, even after stratification by tumor grade or anatomical location.     

31例颅内室管膜瘤术后放射治疗的预后分析

目的 讨论颅内室管膜瘤的临床特点、治疗转归和预后因素。方法 回顾性分析2009年1月-2012年6月收治的31例颅内室管膜瘤术后放射治疗患者的临床资料,男22例,女9例,平均年龄18岁(3~60岁);17例患者行手术全切,14例患者行次全切除;平均放疗剂量53.9 Gy(48.6~60 Gy);观察疗效,分析患者3和5年的疾病无进展时间,总生存时间。对年龄、性别、肿瘤部位、手术切除情况、病理分级、放疗方式、放疗剂量、辅助治疗8个因素进行单因素分析,Cox比例风险模型分析影响预后的因素。结果 中位随访51个月。7例患者术后放疗后复发,5例为原位复发,2例发生全中枢播散。死亡患者6例,其中4例为儿童第Ⅳ脑室间变性室管膜瘤患者。3和5年的无进展生存率(PFS) 分别为80.6% 和75.9%,3和5年的总生存率(OS)分别为 83.9%和76.2%。手术全切组(17/31)和次全切除组(14/31)的3年和5年PFS分别为94.1%和64.3%,90.9%和57.1%,差异均有统计学意义(χ²=4.685、6.311, P<0.05);手术全切组和次全切除组的5年OS分别为83.3%和64.3%,差异有统计学意义(χ²=4.238, P<0.05)。放疗剂量≤55 Gy和>55 Gy两组患者的5年PFS分别为64.2%和100.0%,差异有统计学意义(χ²=4.210, P<0.05)。未观察到严重的不良反应。结论 手术是室管膜瘤的首选治疗方法,部分切除和间变性室管膜瘤患者,术后放疗是最重要的辅助手段。手术切除程度和放疗剂量影响室管膜瘤患者预后。     

Histologic prognostic factors in ependymoma

The prognostic value of a series of histologic signs and clinical features was studied in a series of 298 ependymomas, collected from different institutions. The distribution of tumor sites varied in relation to patient age, with infratentorial cases prevailing under 4 years. Life table univariate analysis demonstrated as highly significant prognostic factors: (1) the number of mitoses; (2) endothelial hyperplasia; (3) necrosis; (4) intracranial site; (5) age <4 years. Multivariate analysis by tumor site revealed mitoses cell density, age >16 years in supratentorial cases, and subependymoma in infratentorial cases to be prognostically important. Comparison of the anaplastic variant with the other tumor types in intracranial cases did not show a significant difference in survival even though the median survival time of anaplastic cases was shorter. The main conclusion is that the histological criteria employed to diagnose anaplasia in gliomas are not useful for recognizing anaplasia in ependymomas. The number of mitoses is a very important prognostic factor in supratentorial cases, whereas endothelial proliferations and necroses are much less important as prognostic factors than in gliomas.     

Intracranial extra-axial ependymoma involving the petroclival region: a rare case report

Intracranial extraaxial ependymomas (IEAEs) are very rare, and extra-axial petroclival ependymoma (EAPE) has not been reported in the reviewed English-language literature by now. We present a male patient with EAPE misdiagnosed as a petroclival meningioma preoperatively, and involved recurrence at 4th months after operation though totally resection. Thereafter, we report this case and discuss the clinical characteristics of the disease by briefly review.     

Prognostic factors for progression-free survival of the filum terminale ependymomas in adults

ObjectiveTo define the prognostic factors for progression and to determine the impact of the histological grading (according to the World Health Organization) on the progression-free survival (PFS) of filum terminale ependymomas.MethodsA retrospective chart review of 38 patients with ependymoma of the filum terminale was performed, focusing on demographic data, preoperative symptoms, tumor size, quality of resection, presence of a tumor capsule, and histological grade.ResultsGross total resection (GTR) was achieved in 30 patients (78.9%). Histopathological analysis found 21 (55.3%) myxopapillary grade I ependymoma (MPE), 16 (42.1%) ependymoma grade II (EGII), and 1 (2.6%) ependymoma grade III. There was no significant difference between the mean ± SD volume of MPE (5840.5 ± 5244.2 mm³) and the one of EGII (7220.3 ± 6305.9 mm³, p = 0.5). The mean ± SD follow-up was 54.1 ± 38.4 months. At last follow-up, 30 (78.9%) patients were free of progression. In multivariate analysis, subtotal resection (p = 0.015) and infiltrative tumor (p = 0.03) were significantly associated with progression. The PFS was significantly higher in patients with encapsulated tumor than in patients with infiltrative tumor (log-rank p = 0.01) and in patients who had a GTR in comparison with those who had an incomplete resection (log-rank p = 0.05). There was no difference in PFS between patient with MPE and EGII (p = 0.1).ConclusionThe progression of ependymoma of the filum terminale highly depends on the quality of resection, and whether the tumor is encapsulated. Except for anaplastic grade, histopathological type does not influence progression.     

Evaluation of NF2 gene deletion in sporadic schwannomas, meningiomas, and ependymomas by chromogenic in situ hybridization

Fluorescence in situ hybridization, loss of heterozygosity testing, and comparative genomic hybridization have been used to detect NF2 gene alterations in both sporadic and neurofibromatosis type 2 (NF2)-associated central nervous system tumors. In this study, we performed chromogenic in situ hybridization (CISH) and immunohistochemistry to evaluate for NF2 gene deletion in a group of sporadic meningiomas, schwannomas, and ependymomas. Twenty-two sporadic tumors, including 9 ependymomas, 10 meningiomas, and 3 schwannomas, were studied. CISH and immunohistochemistry were performed using the NF2 gene deletion probe and NF2 polyclonal antibody. Deletion of the NF2 gene was identified in 11 (50%) tumors, including 60% (6/10) of meningiomas, 33% (3/9) of ependymomas, and 67% (2/3) of schwannomas. The remaining 11 (50%) cases were diploid. Overall, immunoexpression of NF2 protein was observed in 50% (11/22) tumors, and concordance between CISH and immunohistochemistry was observed in 73% of cases. Our results support previous observations that schwannomas and meningiomas, and to a lesser degree, ependymomas, express a high incidence of NF2 gene deletion, which supports the hypothesis that NF2 gene plays an important role in their tumorigenesis. In addition, we have validated CISH as an efficient, economic, and reliable method for routinely assessing NF2 gene deletion in these tumors.