关键词: 朗格汉斯细胞组织细胞增多症, 垂体柄占位, 尿崩症

Abstract: Objective To investigate the clinical feature, diagnosis and therapy of isolated langerhans cell histiocytosis (histiocytosis X) with involvement of a rare site. Methods A case of isolated langerhans cell histiocytosis of the infundibulum in adult was reported. The endocrinic tests, imaging , immunohistochemical and pathological examinations of this case were detected．Through literature review, the pathological and clinical feature, diagnosis , therapy of isolated langerhans cell histiocytosis of the infundibulum were overviewed. Results Magnetic resonance imaging (MRI) of the brain showed a 9-mm homogeneously enhancing mass in the region of the infundibulum. No other lesions were found in other organ systems. The patient underwent an occupying lesion resection of the Infundibulum via right pterion approach. Langerhans cell histiocytosis was diagnosed through pathologic analysis. She was on hormone replacement therapy and close follow-up visit postoperatively Conclusion Isolated langerhans cell histiocytosis of the infundibulum in adult is extremely rare. Understanding of this disease should be deepened to avoid of misdiagnosing and mistreating.

Key words: Langerhans cell histiocytosis, Infundibulum tumor, Diabetes insipidus