Chinese Journal of Contemporary Neurology and Neurosurgery ›› 2020, Vol. 20 ›› Issue (7): 625-631. doi: 10.3969/j.issn.1672-6731.2020.07.012

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Clinical features of Langerhans cell histiocytosis in the sellar region

WANG Zhi-cheng1, ZHU Jian-yu1, ZHANG Yi1, LI Xiao-xu1, LIU Jie1, ZHU Hui-juan2, YAO Yong1, PAN Hui2, DENG Kan1   

  1. 1 Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China;
    2 Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
  • Received:2020-07-17 Online:2020-07-25 Published:2020-07-24
  • Supported by:

    This study was supported by Chinese Academy of Medical Sciences (CAMS) Innovation Fund for Medical Sciences (No. 2016-I2M-1-002).

鞍区朗格汉斯细胞组织细胞增生症临床特点分析

王志成1, 朱建宇1, 张毅1, 李晓旭1, 刘杰1, 朱惠娟2, 姚勇1, 潘慧2, 邓侃1   

  1. 1 100730 中国医学科学院 北京协和医学院 北京协和医院神经外科;
    2 100730 中国医学科学院 北京协和医学院 北京协和医院内分泌科
  • 通讯作者: 邓侃,Email:dcansums@126.com
  • 基金资助:

    中国医学科学院医学与健康科技创新工程重大协同创新项目(项目编号:2016-I2M-1-002)

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

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