儿童孤立性垂体柄朗格汉斯细胞组织细胞增生症的MRI表现

MRI Features of Isolated Langerhans Cell Histiocytosis in Pituitary Stalk in Children

目的探讨孤立性垂体柄朗格汉斯细胞组织细胞增生症(LCH)的临床和MRI表现,提高对本病的认识和诊断水平。资料与方法回顾性分析13例孤立性垂体柄LCH患儿的临床及MRI资料,所有病例均经手术或穿刺活检证实为LCH,均行垂体MRI平扫及增强扫描。结果临床特点:13例患儿均以中枢性尿崩症为首发症状,且均为孤立性垂体柄LCH,临床及影像检查提示未累及其他系统。MRI表现:初次MRI及复查MRI,13例患儿垂体后叶T1WI正常高信号均消失。初次MRI显示,垂体柄呈结节状增粗7例,均匀增粗4例,漏斗部呈结节状增粗2例,动态增强扫描呈渐进性强化、直至明显强化。4例行MRI随访,3例分别于治疗后第6个月、第9个月、第10个月随访MRI基本正常;1例病情反复,治疗后第11个月随访MRI显示病变基本消失,第25个月随访MRI显示病变较第一次明显。结论儿童孤立性垂体柄LCH常以中枢性尿崩症为首发症状,垂体后叶T1WI正常高信号消失,同时有垂体柄增粗,应该考虑本病。MRI随访能很好地监测病灶的变化。

Purpose To explore the clinical and MRI features of isolated Langerhans cell histiocytosis（LCH） in pituitary stalk, to enhance the understanding and improve diagnosis of this disease. Material and Methods Clinical and MRI data of 13 children with isolated LCH in pituitary stalk were retrospectively studied, LCH were confirmed by surgery or biopsy in all cases LCH and all of them underwent pre-and post-contrast MRI scan on pituitary. Results Clinical features： first symptom was central diabetes insipidus in all 13 cases with isolated LCH in pituitary stalk, clinical and imaging examination indicated that no other system was involved. MRI manifestations： on the initial and follow-up MR images, the normal high signal of posterior pituitary of the 13 children subjects on T1 WI all disappeared. MR images showed nodular thickening in the pituitary stalk（n=7） and in infundibulum（n=2）, and homogeneous thickening in the pituitary stalk（n=4）, dynamic enhancement scan showed that the lesions enhanced gradually until obvious enhancement. Follow-up MRI studies were conducted in 4 cases. MRI results in 3 cases appeared basically normal in the sixth, ninth and tenth month during follow-up respectively; 1 cases of recurrent disease, follow-up MRI 11 month after treatment showed lesions disappeared and the 25 months follow-up MRI showed lesions were more obvious than the first scan. Conclusion First symptom of isolated LCH in pituitary stalk in children is central diabetes insipidus, when comes to the loss of hyper-intensity of posterior pituitary and thickening of pituitary stalk, isolated LCH should be considered. MRI follow-up is a good method to monitor the change of the lesion.