位于神经垂体的孤立性朗格汉斯细胞组织细胞增多症，脑脊液中β-人绒毛膜促性腺激素滴度呈阳性。
Solitary Langerhans cell histiocytosis located in the neurohypophysis with a positive titer HCG-β in the cerebrospinal fluid.
作者信息
Kinoshita Yasuyuki, Yamasaki Fumiyuki, Usui Satoshi, Amatya Vishwa Jeet, Tominaga Atsushi, Sugiyama Kazuhiko, Kurisu Kaoru
机构信息
Department of Neurosurgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
Department of Pathology, Graduate School of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
出版信息
Childs Nerv Syst. 2016 May;32(5):901-4. doi: 10.1007/s00381-015-2949-2. Epub 2015 Nov 2.
INTRODUCTION
Beta-human chorionic gonadotropin (HCG-β) is considered to be a useful tumor marker for germ cell tumors (GCTs); however, various tumors other than GCTs, including cystic pituitary adenomas, Rathke's cleft cysts, and craniopharyngiomas, were reported to express HCG-β.
CASE REPORT
We herein present the case of a 5-year-old boy who presented with polyuria and had a solitary lesion in the neurohypophysis with a positive HCG-β titer in the cerebrospinal fluid. Under a preoperative diagnosis of germinoma, a biopsy was performed from the posterior pituitary lobe via the transsphenoidal endoscopic approach and the histological diagnosis was revealed to be Langerhans cell histiocytosis (LCH).
CONCLUSIONS
The finding of a slightly positive HCG-β titer in the cerebrospinal fluid (CSF) cannot exclude the possibility of LCH, and we strongly recommend a histological diagnosis for the diagnosis of a solitary neurohypophysial lesion.
引言
β-人绒毛膜促性腺激素（HCG-β）被认为是生殖细胞肿瘤（GCTs）的一种有用的肿瘤标志物；然而，据报道，除了GCTs之外的各种肿瘤，包括囊性垂体腺瘤、拉克氏囊肿和颅咽管瘤，也表达HCG-β。
病例报告
我们在此报告一例5岁男孩，该男孩表现为多尿，神经垂体有一个孤立性病变，脑脊液中HCG-β滴度呈阳性。在术前诊断为生殖细胞瘤的情况下，通过经蝶窦内镜入路对垂体后叶进行了活检，组织学诊断为朗格汉斯细胞组织细胞增多症（LCH）。
结论
脑脊液（CSF）中HCG-β滴度略呈阳性的发现不能排除LCH的可能性，我们强烈建议对孤立性神经垂体病变进行组织学诊断以明确诊断。
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