Clinical features of Langerhans cell histiocytosis in the sellar region

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 sellarregion. 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 (3patients), followed by both pituitary stalk and hypothalamus (2patients). MRI showed isointensity (8cases) on T1WI, isointensity (6cases), hypointensity (onecase) 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 allpatients. 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.

DOI：10.3969/j.issn.1672⁃6731.2020.07.012