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目的：探讨朗格罕细胞组织细胞增生症累及垂体的MR 表现及相关临床表现。方法：搜集了6 例确诊为朗格罕细胞组织细胞增生症并垂体表现异常的患儿，男5 例，女1 例，年龄2～11 岁，平均（6±3）岁，对其影像及临床表现进行回顾性分析。结果：临床患儿主要以头面部包块，多饮、多尿等就诊。头颅MR 平扫（T1WI）表现6 例患儿神经垂体高信号全部消失，垂体柄增粗5 例，垂体柄著征1 例，垂体饱满1 例，其中3 例治疗后复查垂体及垂体柄均有变化。结论：神经垂体高信号消失，垂体柄增粗或著征为朗格罕细胞组织细胞增生症累及垂体的头颅MR 表现。累及垂体者临床几乎都有多饮、多尿表现。目前，MRI 是诊断朗格罕细胞组织细胞增生症累及垂体的唯一可靠的影像学检查方法，并对治疗后病情随访有重要作用。

英文摘要:

Objective: To study the Langerhans cell histiocytosis invading the pituitary MR findings and associated clinical performance. Methods: We collected six patients who were diagnosed as Langerhans cell histiocytosis and pituitary expression abnormalities in children, 5 males, 1 female, aged from 2 to 11 years, average age (6 ±3). Their images findings and clinical performance were taken as a retrospective analysis. Results: The main diagnosed reason for children was that they got head and face mass, polydipsia, urine. At the same time, we toke MR scan (T1W1) and found that the high signal of neurohypophysis disappeared, unfounded in six cases, pituitary stalk thickened in five cases, pituitary stalk distinctness in one case, pituitary plumped in one case, and the signal of pituitary and pituitary stalk all changed by MR scan retest after treatment. Conclusion: Pituitary high signal disappears, pituitary stalk thickening or distinctness, are the MR imaging performance for the Langerhans cell histiocytosis invading brain. Polydipsia and polyuria will almostly appear if pituitary is invaded by the Langerhans cell histiocytosis. Currently, MRI is the only reliable imaging diagnosis for the Langerhans cell histiocytosis invading the pituitary, and has an important role for follow-up.

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