Isoo A, Ueki K, Ishida T, Yoshikawa T, Fujimaki T, Suzuki I, Sasaki T, Kirino T
Department of Neurosurgery, University of Tokyo Hospital, Tokyo.
Neurol Med Chir (Tokyo). 2000 Oct;40(10):532-5. doi: 10.2176/nmc.40.532.
Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. Biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.
朗格汉斯细胞组织细胞增多症很少表现为垂体 - 下丘脑区域的孤立性病变，且与生殖细胞瘤难以区分，后者更为常见，尤其是在日本人中。一名14岁女孩和一名9岁女孩以烦渴和多尿为首发症状。磁共振（MR）成像显示垂体柄处有一个圆形肿块，在T1加权成像上呈等信号，在T2加权成像上呈高信号。内分泌检查显示轻度垂体功能减退伴中枢性尿崩症。两名患者均接受了开颅手术。组织学检查显示有肉芽肿组织，伴有嗜酸性粒细胞浸润和频繁的朗格汉斯组织细胞聚集，符合朗格汉斯细胞组织细胞增多症的诊断。给予20 Gy的低剂量局部照射。第一名患者随访8年，其垂体功能减退逐渐改善至最低水平，仅残留闭经症状。最近的MR成像显示该区域无残留肿块。第二名患者随访15个月，其尿崩症病情稳定。治疗后5个月进行的MR成像显示肿块明显缩小。这些病例再次强调了组织学诊断对于具有相似神经影像学表现的病变的重要性。活检和低剂量照射是治疗这种罕见且本质上为良性病变的有效方法，而不是试图完全切除肿块。
