Vitanovics Dusán, Áfra Dénes, Nagy Gábor, Hanzely Zoltán, Turányi Eszter, Banczerowski Péter
Ideggyogy Sz. 2014 Nov 30;67(11-12):415-9.
Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. PATIENT SELECTION AND METHODS: Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005.
Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years.
Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.
脑室内室管膜下瘤是罕见的良性肿瘤,常被误诊为室管膜瘤。旨在回顾室管膜下瘤的临床病理特征。
对脑室内室管膜下瘤进行回顾性临床分析,并对1985年至2005年间在本中心接受手术的组织学切片进行系统回顾。
20例室管膜下瘤患者,中位年龄50岁(范围19 - 77岁)。第三脑室2例(10%),第四脑室3例(15%),侧脑室15例。男性居多(12例对8例)。共济失调(n = 13)和视乳头水肿(n = 7)是最常见的临床表现。15例患者接受了全切除,5例接受了次全切除。所有病例均未显示有丝分裂象、血管内皮细胞增殖或坏死。细胞增殖标志物MIB - 1活性(阳性染色肿瘤细胞百分比)范围为0至1.4%(平均0.3%)。2例在CT时代之前接受了术前放射治疗(50 Gy),另外3例患者因最初组织学诊断为低级别室管膜瘤而接受了术后放射治疗。3例患者(15%)在术后1至3个月死于手术并发症,3例患者在8、26和110个月死于无关原因。15例患者存活,中位随访时间10年,无肿瘤复发迹象。
室管膜下瘤是低级别病变,患者无需辅助放疗预后良好。来自细胞较多区域的小样本可能与低级别室管膜瘤混淆,进而可能进行不必要的放疗。复发、快速生长率应进行组织学复查,因为室管膜瘤细胞少的区域也可能造成混淆。