作者：伍发，杨钰林，王鹏，杜飞舟，李建浩，唐晨程，云海龙，王新伟，蒋锐

单位：

1.610083四川省成都市，西部战区总医院放射诊断科　 2.611100四川省成都市第五人民医院超声科 3.610083四川省成都市，西部战区总医院病理科　 4.610011四川省成都市公共卫生临床医疗中心放射科 通信作者：蒋锐，E-mail：jiangrui07@sina.com

Units：

1.Department of Radiology, Western Theater General Hospital, Chengdu 610083, China 2.Department of Ultrasound, Chengdu 5th People's Hospital, Chengdu 611100, China 3.Department of Pathology, Western Theater General Hospital, Chengdu 610083, China 4.Department of Radiology, Chengdu Public Health Clinical Medical Center, Chengdu 610011, China Corresponding author: JIANG Rui, E-mail: jiangrui07@sina.com

关键词：

脑膜瘤; 透明细胞型脑膜瘤; 症状和体征; 磁共振成像; 病理学;

Keywords：

Meningioma; Clear cell meningioma; Symptoms and signs; Magnetic resonance imaging; Pathology

CLC：

DOI：

10.12114/j.issn.1008-5971.2022.00.286

Funds：

四川省科技计划项目（2018JY0604）；四川省医学会（恒瑞）科研基金专项科研课题（2021HR75）；西部战区总医 院助推基金（2019ZT09）；西部战区总医院院管课题（2021-XZYG-C04，2021-XZYG-C05）

摘要：

目的 总结透明细胞型脑膜瘤(CCM)的临床、MRI及病理特征，以提高临床医师对该病术前诊断的准确率。方法 回顾性收集2015年1月至2022年5月在西部战区总医院收治的经病理检查确诊的CCM患者24例为研究对象，收集患者基线资料(性别、年龄)、临床资料(首发症状、术前KPS评分、脑膜瘤切除程度的Simpson分级、肿瘤是否侵犯周围结构、术后是否采用放疗、术后并发症及复发情况)、MRI检查结果(肿瘤大小、肿瘤位置、肿瘤性质及脑膜尾征、分叶征、黑环征、囊变坏死发生情况)及病理结果。根据肿瘤大小将患者分为小肿瘤组(肿瘤最大直径<4.0 cm)12例和大肿瘤组(肿瘤最大直径≥4.0 cm)12例，比较两组脑膜尾征、分叶征、黑环征、囊变坏死发生率。结果 24例患者中，男8例，女16例；年龄15～68岁，平均(44.6±15.4)岁；首发症状：以头晕、头痛为主要表现20例，以视力下降为主要表现2例，以乏力为主要表现2例；术前KPS评分70～90分，平均(87.1±6.2)分；Simpson分级：Ⅰ级17例，Ⅱ级5例，Ⅲ级2例；肿瘤侵犯周围结构8例；术后采用放疗3例；术后出现并发症9例；术后随访6～18个月，复发6例。肿瘤最大直径0.8～6.4 cm；肿瘤位于桥小脑角区10例，位于大脑凸面9例，靠近静脉窦生长2例，位于鞍区1例，位于后颅窝1例，颅内多发1例；肿瘤性质：实性13例，囊实性11例；出现脑膜尾征20例，出现分叶征4例，出现黑环征13例，出现囊变坏死11例。两组脑膜尾征、分叶征发生率比较，差异无统计学意义(P>0.05)；大肿瘤组黑环征发生率低于小肿瘤组，囊变坏死发生率高于小肿瘤组(P<0.05)。病理检查结果显示，肿瘤组织呈多角形，胞质透明，血管周围和间质胶原纤维丰富，旋涡状结构呈灶性分布，肿瘤内部分布丰富的结缔组织纤维带。结论 CCM属于脑膜瘤中罕见亚型，其临床及MRI表现有一定特点，肿瘤大小与MRI表现相关，小肿瘤更易发生黑环征，大肿瘤更易发生囊变坏死。MRI对CCM诊断有一定帮助，但确诊还需依靠病理检查。

Abstract：

【Abstract】 Objective To summarize the clinical, MRI and pathological features of clear cell meningioma (CCM) , and to improve the accuracy of preoperative diagnosis. Methods Twenty-four patients with CCM diagnosed by pathology admitted to the Western Theater General Hospital from January 2015 to May 2022 were retrospectively collected as the research subjects. The baseline data (gender, age) , clinical data (initial symptoms, preoperative KPS score, Simpson grade of meningioma resection degree, whether the tumor invades surrounding structures, whether use radiotherapy after surgery, postoperative complications and recurrence) , MRI examination results (tumor size, tumor location, tumor nature, and incidence of meningeal tail sign, lobulation sign, black ring sign, cystic necrosis) and pathological results were collected. The patients were divided into small tumor group (maximum diameter of tumor < 4.0 cm) (n=12) and large tumor group (maximum diameter of tumor ≥ 4.0 cm) (n=12) according to the tumor size. The incidence of meningeal tail sign, lobulation sign, black ring sign, cystic necrosis were compared between the two groups. Results Among the 24 patients, there were 8 males and 16 females; the age ranged from 15 to 68 years old, with an average of (44.6±15.4) years old; the first symptoms: dizziness and headache in 20 cases, vision loss in 2 cases and fatigue in 2 cases. The preoperative KPS score ranged from 70 to 90, with an average of (87.1±6.2) ; Simpson classification: grade Ⅰ in 17 cases, grade Ⅱ in 5 cases, and grade Ⅲ in 2 cases; tumor invaded surrounding structures in 8 cases; postoperative radiotherapy was used in 3 cases; postoperative complications occurred in 9 cases; and 6 cases recurred after 6-18 months' follow-up. The maximum diameter of tumor was 0.8-6.4 cm; tumors located in the cerebellopontine angle in 10 cases, located in the convex surface of the brain in 9 cases, grew close to the venous sinus in 2 cases, located in the sellar area in 1 case, located in the posterior cranial fossa in 1 case, and were multiple intracranial in 1 case; tumor nature: solid in 13 cases, cystic solid in 11 cases. Among the 24 patients, 20 cases showed meningeal tail sign, 4 cases showed lobulated sign, 13 cases showed black ring sign, 11 cases showed cystic necrosis. There was no significant difference in the incidence of meningeal tail sign and lobulation sign between the two groups (P > 0.05) ; the incidence of black ring sign in the large tumor group was lower than that in the small tumor group, and the incidence of cystic necrosis was higher than that in the small tumor group (P < 0.05) . The pathological results showed that the tumor tissue was polygonal, with transparent cytoplasm, abundant perivascular and interstitial collagen fibers, swirled structure with focal distribution, and abundant connective tissue fibrous bands inside the tumor. Conclusion CCM is a rare subtype of meningiomas, and its clinical features and MRI manifestations have certain characteristics. The size of the tumor is related to the MRI manifestations. Small tumors are more prone to black ring sign, and large tumors are more prone to cystic necrosis. MRI is helpful for the diagnosis of CCM, but the diagnosis still depends on pathology.

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