Isolated hypothalamic-pituitary langerhans’ cell histiocytosis in female adult
Isolated hypothalamic-pituitary langerhans’ cell histiocytosis in female adult
复制标题
女性成人孤立性下丘脑-垂体朗格汉斯细胞组织细胞增多症
DOI：
10.1097/md.0000000000013853
复制
发表时间：
2019
期刊：
影响因子：
1.6
通讯作者：
Jianwei Li
中科院分区：
文献类型：
--
作者：
Huiwen Tan;Kai Yu;Yerong Yu;Zhengmei An;Jianwei Li
原理：朗格汉斯细胞组织细胞增生症（LCH）以未成熟树突状细胞的克隆性增殖为特征，主要累及儿童。成人鞍区LCH极为罕见。在文献中，最佳的治疗方法仍不清楚，只有少数病例的LCH使用手术治疗。在这里，我们提出了一个罕见的情况下，孤立的下丘脑-垂体LHC的女性成年人。我们着重阐述LCH的临床表现和免疫组化特征，并探讨成人LCH的合理治疗方法。患者问题：一位50岁女性因多饮多尿3个月入院。诊断：影像学检查显示垂体后叶病变（0.5 × 0.9 × 0.4 cm），垂体柄增大，鞍区增强MRI中度强化。 病理切片中郎格罕氏细胞S-100蛋白和Ki-67抗原标记阳性，足以诊断中枢神经系统LCH。干预措施：鞍区局限性LCH患者接受手术和化疗。诊断为垂体病变所致中枢性尿崩症后行伽玛刀治疗。5年后行肿瘤切除术。但在2017年最新一次MRI中，结节阴影变大（约1.4 cm），给予化疗和进一步全身治疗。 结局：12个月随访时，未发现局部复发。经验教训：对于LCH，虽然难以诊断，也没有明确的成人标准治疗方法，但如果有神经症状或组织学诊断，应考虑手术。目前的研究表明，当中枢神经系统（CNS）受累时，某些表现可能是有意义的。对于鞍区复杂疾病，多学科团队（MDT）的诊断和治疗模式应有助于获得更好的临床疗效。
Rationale: Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation of immature dendritic cells, mainly affects children. LCH in adult sellar region is extremely rare. In literature, optimal treatments remain unclear and only a few cases of LCH were treated using surgery. Here, we present a rare case of isolated hypothalamic-pituitary LHC in female adult. We focused on elucidating the clinical manifestations and immunohistochemical features of LCH, and exploring the proper treatment in adults. Patient concerns: A 50-year-old woman was admitted to our hospital, presenting with polydipsia and polyuria for over 3 months. Diagnoses: Radiological studies revealed lesions (0.5 × 0.9 × 0.4 cm) on posterior pituitary and enlarged pituitary stalk, which was moderately enhanced on contrast magnetic resonance imaging (MRI) of sellar region. In biopsy, pathological examination of Langerhans cells were observed with positive S-100 protein and Ki-67 antigen markers, findings were sufficient to establish a diagnosis of central nervous system (CNS) LCH. Interventions: The patient with LCH restricted in the sellar region received both surgery and chemotherapy. Gamma knife radiosurgery was performed after diagnosed as central diabetes insipidus (CDI) induced by pituitary lesion. And tumorectomy was performed 5 years later. However, in the latest MRI in 2017, the nodular shadow became larger (about 1.4 cm), chemotherapy and further systemic therapy were given. Outcomes: At 12-month follow-up, no local reoccurrence was noticed. Lessons: For LCH, though difficult to be diagnosed and none defined standard therapeutic approach to adults, surgery should be considered if there are neurological symptoms or histological diagnosis. The present study showed that some manifestations can be meaningful when central nervous system (CNS) is involved. For complex diseases in the sellar region, multi-disciplinary team (MDT) model of diagnosis and treatment should be helpful for better clinical efficacy.