Tamai Sho, Ueno Megumi, Hayashi Yasuhiko, Sasagawa Yasuo, Watanabe Takuya, Murakami Ken-Ichi, Nakada Mitsutoshi, Hayashi Yutaka
Department of Neurosurgery, Ishikawa Prefectural Central Hospital, Kanazawa, Japan.
Department of Neurosurgery, Kanazawa University, Kanazawa, Japan.
Surg Neurol Int. 2018 Oct 3;9:197. doi: 10.4103/sni.sni_229_18. eCollection 2018.
Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system; it is caused by dendritic cell proliferation, and typically occurs in children. LCH frequently appears in the pituitary stalk and rarely results in multiple enhanced lesions in the brain parenchyma.
We present a case of a 40-year-old woman who deveolped panhypopituitarism and central diabetes insipidus in the postpartum period requiring hormone replacement therapy. At first, magnetic resonance imaging only revealed thickening of the pituitary stalk; while 6 months later, a single enhanced mass lesion was detected in the hypothalamus. Another 5 months later, the lesion had enlarged with appearance of multiple, enhanced satellite lesions in the basal ganglia and white matter. The patient underwent successful craniotomy to obtain a biopsy sample; LCH of the hypothalamus was definitively diagnosis by histopathological examination. Steroids were administrated and resulted in significant reduction of all lesions.
Definitive histopathological diagnosis and subsequent appropriate therapy, such as steroid administration, are required when LCH lesions in the hypothalamus become progressively enlarged and new lesions appear in the brain parenchyma.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见疾病，可能累及中枢神经系统；它由树突状细胞增殖引起，通常发生于儿童。LCH常出现在垂体柄，很少导致脑实质内多发强化病灶。
我们报告一例40岁女性病例，其在产后出现全垂体功能减退和中枢性尿崩症，需要激素替代治疗。起初，磁共振成像仅显示垂体柄增粗；6个月后，在下丘脑发现一个单发强化肿块病灶。又过了5个月，病灶增大，在基底节和白质出现多个强化卫星病灶。患者接受了成功的开颅手术以获取活检样本；通过组织病理学检查确诊为下丘脑LCH。给予类固醇治疗后，所有病灶显著缩小。
当下丘脑LCH病灶逐渐增大且脑实质出现新病灶时，需要进行明确的组织病理学诊断并随后进行适当治疗，如给予类固醇治疗。
