1例成人垂体柄孤立性朗格汉斯细胞组织细胞增多症

Langerhans cell histiocytosis of the infundibulum in adult: a case report

目的探讨罕见孤立部位受累的朗格汉斯细胞组织细胞增多症(组织细胞增多症X)的临床特点、诊治方法。方法报道1例成人孤立性垂体柄受累的朗格汉斯细胞组织细胞增多症,通过内分泌学、影像学、病理组织学、免疫组织化学方法的实验室检查,结合文献复习,探讨其病理、临床、诊断、治疗特点。结果患者头颅MR I示为垂体柄区域直径9 mm占位性病变,能够均匀强化。相关检查显示无其他系统、部位受累。进行了经右侧翼点入路、垂体柄区占位性病变切除术。病理诊断为朗格汉斯细胞组织细胞增多症。术后予以激素替代治疗,密切随访中。结论孤立性成人垂体柄朗格汉斯细胞组织细胞增多症极为罕见,应加深对其的认识以避免漏诊和误诊。

Objective To investigate the clinical feature, diagnosis and therapy of isolated Langerhans cell histiocytosis （ histiocytosis X） with unusual localization. Methods A case of isolated Langerhans cell histiocytosis of the infundibulum 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 lesion was 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 improved to avert misdiagnosing.