鞍区朗格汉斯细胞组织细胞增生症临床特点分析

doi:10.3969/j.issn.1672-6731.2020.07.012

摘要/Abstract

摘要：

目的总结鞍区朗格汉斯细胞组织细胞增生症的临床特点，探讨鞍区病变组织活检术的手术指征和安全性。方法与结果 2011年11月至2019年11月通过神经内镜下经鼻蝶入路或扩大经鼻蝶入路鞍区病变组织活检术明确诊断的朗格汉斯细胞组织细胞增生症患者共8例，男女比例1 ∶ 1，中位年龄15.50岁，均以中枢性尿崩症发病，伴有腺垂体功能减退症状（7例）；病变累及范围主要包括垂体柄（7例次）、鞍内（5例次）和下丘脑（3例次），鞍内和垂体柄同时受累（3例）较为常见，其次为垂体柄和下丘脑（2例）；MRI主要表现为T₁WI等信号（8例），T₂WI等信号（6例）、低信号（1例）和混杂信号（1例），呈均匀强化（6例）或不均匀强化（2例），未见T₁WI神经垂体高信号。术后1个月内仅1例出现短暂性下丘脑反应，无一例出现脑脊液鼻漏、中枢神经系统感染、非计划二次手术、死亡等其他不良事件。结论鞍区朗格汉斯细胞组织细胞增生症的临床表现缺乏特异性，应注意与垂体腺瘤、生殖细胞肿瘤、淋巴细胞性垂体炎等鞍区病变相鉴别。组织活检术仍为确诊“金标准”，神经内镜下经鼻蝶入路鞍区病变组织活检术安全、可靠。

关键词: 组织细胞增多症, 朗格尔汉斯细胞, 蝶鞍, 垂体肿瘤, 内窥镜检查, 活组织检查

Abstract:

Objective To summarize the clinical characteristics of Langerhans cell histiocytosis (LCH) in sellar region, to explore the indication and safety of pathological biopsy in sellar region. Methods and Results Eight patients with LCH who underwent biopsy by endoscopic transsphenoidal approach from November 2011 to November 2019 were retrospectively analyzed. The ratio of male to female was 1:1. The median age of 8 patients was 15.50 years old. All patients had central diabetes insipidus as the inaugural manifestation, and 7 patients had hypothalamus-related symptoms. Lesions in 7 patients involved the pituitary stalk, and in 5 patients involved the intrasellar region. The most common site of involvement was both intrasellar region and pituitary stalk (3 patients), followed by both pituitary stalk and hypothalamus (2 patients). MRI showed isointensity (8 cases) on T₁WI, isointensity (6 cases), hypointensity (one case) and heterogeneous intensity (one case) on T₂WI, while 6 with homogeneous enhancement and 2 with heterogeneous enhancement. The hyperintensity on T₁WI of posterior pituitary was absent in all patients. The posterior pituitary bright spot was absent in all patients. Only one patient had a transient hypothalamic dysfunction after operation. None patient had cerebrospinal fluid leakage, central nervous system infection, unplanned secondary operation, death and other adverse events within one month after operation. Conclusions There were no specific clinical manifestations for LCH in sellar region, and this disease should be considered to differentiate from pituitary adenomas, germ cell tumor and lymphocytic hypophysitis. Biopsy was considered as "gold standard" for diagnosis. And biopsy by endoscopic transsphenoidal approach was a safe and reliable way for confirmation.

Key words: Histiocytosis, Langerhans-cell, Sellar turica, Pituitary neoplasms, Endoscopy, Biopsy

王志成, 朱建宇, 张毅, 李晓旭, 刘杰, 朱惠娟, 姚勇, 潘慧, 邓侃. 鞍区朗格汉斯细胞组织细胞增生症临床特点分析[J]. 中国现代神经疾病杂志, 2020, 20(7): 625-631.

WANG Zhi-cheng, ZHU Jian-yu, ZHANG Yi, LI Xiao-xu, LIU Jie, ZHU Hui-juan, YAO Yong, PAN Hui, DENG Kan. Clinical features of Langerhans cell histiocytosis in the sellar region[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2020, 20(7): 625-631.

http://journal11.magtechjournal.com/cjcnn/CN/Y2020/V20/I7/625

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