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室管膜下瘤:14例肿瘤的临床病理研究,包括与其他室管膜肿瘤的MIB-1免疫组化对比分析。

Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms.

作者信息

Prayson R A, Suh J H

机构信息

Department of Anatomic Pathology, Cleveland Clinic Foundation, Ohio 44195, USA.

出版信息

Arch Pathol Lab Med. 1999 Apr;123(4):306-9. doi: 10.5858/1999-123-0306-S.

Abstract

BACKGROUND

Subependymomas are uncommonly encountered ependymal tumors, which are important to distinguish from ordinary ependymomas because of their generally better prognosis.

OBJECTIVE

To review the clinicopathologic features and MIB-1 labeling indices (marker of cell proliferation) of 14 subependymomas.

DESIGN

Retrospective review of 14 subependymomas encountered in a tertiary care setting.

RESULTS

Fourteen ependymomas presenting in 8 men and 6 women between the ages of 18 and 78 years (mean, 53.6 years) comprise the study group. The most common clinical presentations included ataxia (n = 4), dizziness/vertigo (n = 3), nausea/vomiting (n = 3), headaches (n = 3), and incidental finding at autopsy (n = 2). Tumor locations included fourth ventricle (n = 7), lateral ventricle (n = 4), third ventricle (n = 2), and thoracic spinal cord (n = 1). Eight patients underwent gross total resection, and 4 had subtotal resection. Tumors were characterized by clustering of cell nuclei arranged against a fibrillary background. Focal cystic degeneration was seen in 10 tumors, hemosiderin deposition in 8 tumors, sclerotic vessels in 8 tumors, calcifications in 5 tumors, and focal nuclear pleomorphism in 2 tumors. Mitotic figures, vascular endothelial proliferation, and necrosis were not seen in any of these tumors. Cell proliferation marker MIB-1 labeling indices (percentage of positive staining tumor cells) ranged from 0 to 1.4 (mean, 0.3). In comparison, 13 myxopapillary ependymomas had labeling indices ranging from 0 to 5.5 (mean, 1.1). Thirty-nine low-grade ependymomas had MIB-1 labeling indices of 0.1 to 5.4 (mean, 1.1). Fourteen anaplastic/malignant ependymomas had MIB-1 labeling indices ranging from 0.4 to 34.0 (mean, 12.8). One subependymoma was treated with radiation therapy. Six patients were alive with no evidence of tumor at a mean follow-up of 94.4 months. Two patients were alive with residual tumor (follow-up of 4 and 53 months). Two patients died with tumor at 0.67 and 43.4 months. One patient was lost to follow-up, 1 is a recent case, and 2 were incidental findings at autopsy. None of the patients developed tumor recurrence.

CONCLUSIONS

Subependymomas are generally low-grade lesions, as evidenced by their benign clinical course and low MIB-1 labeling indices. Compared with other ependymal tumors, subependymomas have the lowest rate of cell proliferation as evidenced by MIB-1 immunostaining.

摘要

背景

室管膜下瘤是一种罕见的室管膜肿瘤,因其预后通常较好,所以与普通室管膜瘤相鉴别很重要。

目的

回顾14例室管膜下瘤的临床病理特征及MIB-1标记指数(细胞增殖标志物)。

设计

对在三级医疗中心遇到的14例室管膜下瘤进行回顾性研究。

结果

研究组包括14例室管膜下瘤患者,其中男性8例,女性6例,年龄在18至78岁之间(平均53.6岁)。最常见的临床表现包括共济失调(4例)、头晕/眩晕(3例)、恶心/呕吐(3例)、头痛(3例)以及尸检时偶然发现(2例)。肿瘤位置包括第四脑室(7例)、侧脑室(4例)、第三脑室(2例)和胸段脊髓(1例)。8例患者接受了肿瘤全切术,4例接受了次全切除术。肿瘤的特征是细胞核在纤维背景上呈簇状排列。10例肿瘤可见局灶性囊性变,8例有含铁血黄素沉积,8例有硬化血管,5例有钙化,2例有局灶性核异型性。这些肿瘤均未见有丝分裂象、血管内皮细胞增殖及坏死。细胞增殖标志物MIB-1标记指数(阳性染色肿瘤细胞的百分比)范围为0至1.4(平均0.3)。相比之下,13例黏液乳头型室管膜瘤的标记指数范围为0至5.5(平均1.1)。39例低级别室管膜瘤的MIB-1标记指数为0.1至5.4(平均1.1)。14例间变性/恶性室管膜瘤的MIB-1标记指数范围为0.4至34.0(平均12.8)。1例室管膜下瘤接受了放射治疗。6例患者存活,平均随访94.4个月,无肿瘤证据。2例患者存活但有残留肿瘤(随访时间分别为4个月和53个月)。2例患者分别于0.67个月和43.4个月死于肿瘤。1例患者失访,1例为近期病例,2例为尸检时偶然发现。所有患者均未发生肿瘤复发。

结论

室管膜下瘤通常为低级别病变,其良性的临床病程及低MIB-1标记指数可证明这一点。与其他室管膜肿瘤相比,MIB-1免疫染色显示室管膜下瘤的细胞增殖率最低。

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