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Analysis of Clinical Characteristics and Biopsy Safety of Indistinguishable Lesions in the Sellar Area
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摘要:
目的 总结蝶鞍区疑难病变的临床特点, 探讨神经内镜下经鼻蝶窦鞍区病变活检术的临床价值及安全性。 方法 回顾性收集2011年7月1日至2019年7月1日于北京协和医院神经外科接受神经内镜下经鼻蝶窦入路(endoscopic transsphenoidal approach, ETA)鞍内占位活检术或神经内镜下扩大经鼻蝶窦入路(endoscopic extended transsphenoidal approach, EETA)鞍上占位活检术的所有蝶鞍区病变患者临床资料, 分析其病因构成、临床表现、实验室检查、影像学表现及术后并发症情况。 结果 共142例符合纳入标准的患者入选本研究, 其中115例(81.0%, 115/142)接受ETA鞍内占位活检术, 27例(19.0%, 27/142)接受EETA鞍上占位活检术。142例蝶鞍区病变的病因包括囊性病变12例(8.5%, 12/142)、炎症/感染性疾病30例(21.1%, 30/142)和肿瘤100例(70.4%, 100/142), 最常见的诊断依次是生殖细胞肿瘤(79例)、朗格汉斯细胞组织细胞增生症(8例)、Rathke's囊肿(8例)和淋巴细胞垂体炎(7例)。142例患者中, 最常见的临床表现为中枢性尿崩相关症状; 无脑脊液甲胎蛋白升高; 29例脑脊液β人绒毛膜促性腺激素升高, 其中27例(93.1%)为生殖细胞肿瘤, 2例(6.9%)为颅咽管瘤; 肿瘤和炎症/感染性疾病最常同时累及鞍内和垂体柄(25.0%和33.3%), 其次为鞍内或由鞍内延伸至鞍上(21.0%和30.0%), 囊性病变则最常累及鞍内/由鞍内延伸至鞍上(75.0%)。97.0%病例的头颅MRI表现为垂体后叶亮斑缺失。活检术后1个月内, 7例(4.9%, 7/142)患者出现中枢神经系统感染, 3例(2.1%, 3/142)出现脑脊液漏(其中2例行二次手术修补后恢复), 3例(2.1%, 3/142)出现眼球运动功能障碍, 2例(1.4%, 2/142)出现视力下降, 2例(1.4%, 2/142)出现视野缺损或较术前加重, 经治疗后均基本恢复正常。 结论 蝶鞍区病变临床表现常缺乏特异性, 组织病理学检查是诊断的金标准。ETA鞍内和EETA鞍上占位活检术安全可靠。 Abstract:Objective The aim of this study was to summarize the etiology and clinical characteristics ofpatients with indistinguishable sellar lesions, and to explore the clinical value and safety of biopsy by endoscopic trans-sphenoidal approach. Methods The clinical data were retrospectively collected and analyzed in patients with indistinguishable sellar lesions who underwent biopsy by the endoscopic trans-sphenoidal approach (ETA) or endoscopic extended trans-sphenoidal approach (EETA) in Peking Union Medical College Hospital from July 1, 2011 to July 1, 2019, including the etiology, clinical manifestations, laboratory examinations, radiological features, and postoperative complications. Results A total of 142 patients who met the inclusive criteria were enrolled in this study. ETA biopsy was performed in 115 patients (81.0%, 115/142) and EETA in 27 patients (19.0%, 27/142). The etiology included:cystic lesions in 12 patients (8.5%, 12/142), inflammatory/infectious diseases in 30 patients (21.1%, 30/142), and tumors in 100 patients (70.4%, 100/142). The most common diseases were germ cell tumors (79 patients), Langerhans cell histiocytosis (8 patients), Rathke's cysts (8 patients), and lymphocytic hypophysitis (7 patients). The clinical manifestations of patients with this type of disease frequently lacked specificity. Among the 142 patients, the most common manifestations were those related to central diabetes insipidus. There were no patients with elevated alpha-fetoprotein in the cerebrospinal fluid and 29 patients with elevated beta-human chorionic gonadotropin in the cerebrospinal fluid. Among these 29 patients, 27 (93.1%) were germ cell tumors and 2 (6.9%) were craniopharyngiomas. Tumors and inflammatory/infectious diseases most often involved both the sella and pituitary stalk (25.0%, 33.3%), followed by the sella or protruding from the sella (21.0%, 30.0%). Cystic lesions most often involved the sella or protruding from the sella (75.0%). The disappearance of bright spots in the posterior pituitary was discovered in 97.0% of patients. Within 1 month after the biopsy, 7 patients (4.9%, 7/142) had central nervous system infection; 3 patients (2.1%, 3/142) had cerebrospinal fluid leakage with 2 of them recovered after surgical repair. Three patients (2.1%, 3/142) had worsened eye movement; 2 (1.4%, 2/142) had worsened visual acuity; 2 (1.4%, 2/142) had a decreased visual field, who have recovered basically after treatment. Conclusions The clinical characteristics of lesions in the sellar area frequently lack specificity. Histopathological examination is the gold standard of diagnosis. Endoscopic trans-sphenoidal biopsy in the sellar area is safe and reliable. 利益冲突 无 -
表 1 142例蝶鞍区疑难病变患者的病因构成及一般临床资料
病因 男/女(n) 年龄[M(P25, P75)] 诊断 例数(%) 男/女(n) 年龄[M(P25, P75)] 囊性病变(n=12) 4/8 37.0(19.3，52.8) Rathke's囊肿 8(5.6) 2/6 37.0(27.0, 49.5) 垂体囊肿 4(2.8) 2/2 36.0(9.5, 56.5) 炎症/感染性疾病(n=30) 13/17 34.5(17.8，47.8) 朗格汉斯细胞组织细胞增生症 8(5.6) 4/4 15.5(12.3, 38.0) 淋巴细胞垂体炎 7(4.9) 3/4 33.0(32.0, 39.0) 垂体炎症 5(3.5) 2/3 25.0(17.0, 46.0) IgG4相关垂体炎 2(1.4) 1/1 47.5(41.0, 54.0) 肉芽肿性垂体炎 1(0.7) 0/1 - Tolosa-Hunt综合征 1(0.7) 1/0 - 垂体脓肿 6(4.2) 2/4 45.0(33.0, 56.0) 肿瘤(n=100) 36/64 16.0(11.0，24.8) 生殖细胞瘤 77(54.2) 28/49 14.0(10.0, 21.0) 混合型生殖细胞肿瘤 1(0.7) 1/0 - 畸胎瘤 1(0.7) 0/1 - 颅咽管瘤 7(4.9) 1/6 26.0(14.0, 43.0) 胶质瘤 4(2.8) 2/2 16.0(12.3, 35.5) 转移性恶性肿瘤 6(4.2) 2/4 60.5(45.8, 67.3) 垂体细胞瘤 1(0.7) 1/0 - 脊索瘤 1(0.7) 1/0 - 颗粒细胞瘤 1(0.7) 0/1 - 鞍区癌样分化不良型脑膜瘤 1(0.7) 0/1 - 
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表 2 142例蝶鞍区疑难病变患者的临床症状[n(%)]
症状 总体(n=142) 囊性病变(n=12) 炎症/感染性疾病(n=30) 肿瘤(n=100) 尿崩 124(87.3) 11(91.7) 28(93.3) 85(85.0) 视力下降 40(28.2) 4(33.3) 6(20.0) 30(30.0) 视野缺损 29(20.4) 3(25.0) 5(16.7) 21(21.0) 眼球运动功能障碍 5(3.5) 0(0) 2(6.7) 3(3.0) 头痛 47(33.1) 3(25.0) 10(33.3) 34(34.0) 生长迟缓 22(15.5) 1(8.3) 0(0) 21(21.0) 下丘脑相关症状 6(4.2) 0(0) 3(10.0) 3(3.0) 垂体前叶功能低下相关症状 100(70.4) 7(58.3) 21(70.0) 72(72.0) 其他 10(7.0) 3(25.0) 1(3.3) 6(6.0)
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表 3 142例蝶鞍区疑难病变患者的头颅MRI表现[n(%)]
影像学特点 总体(n=142) 囊性病变(n=12) 炎症/感染性疾病(n=30) 肿瘤(n=100) 受累部位 鞍内/鞍内延伸至鞍上 39(27.5) 9(75.0) 9(30.0) 21(21.0) 垂体柄 11(7.7) 0(0) 3(10.0) 8(8.0) 下丘脑 2(1.4) 0(0) 0(0) 2(2.0) 鞍内+垂体柄 37(26.1) 2(16.7) 10(33.3) 25(25.0) 垂体柄+下丘脑 15(10.6) 0(0) 5(16.7) 10(10.0) 鞍内+垂体柄+下丘脑 16(11.3) 1(8.3) 1(3.3) 14(14.0) 其他 22(15.5) 0(0) 2(6.7) 20(20.0) 垂体后叶亮斑缺失* 130(97.0) 10(100.0) 27(93.1) 93(97.9) *部分患者MRI资料不完整，以可获取资料数量进行统计
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表 4 142例蝶鞍区病变患者术后1个月内并发症发生情况[n (%) ]
并发症 总体
(n=142)ETA鞍内占位
活检(n=115)EETA鞍上占位
活检(n=27)脑脊液漏 3(2.1) 1(0.9) 2(7.4) 中枢神经系统感染 7(4.9) 2(1.7) 5(18.5) 视力较术前下降 2(1.4) 1(0.9) 1(3.7) 视野缺损或较术前加重 2(1.4) 1(0.9) 1(3.7) 眼球活动功能障碍 3(2.1) 1(0.9) 2(7.4) ETA:神经内镜下经鼻蝶窦入路；EETA:神经内镜下扩大经鼻蝶窦入路
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