Kinoshita Yasuyuki, Yamasaki Fumiyuki, Usui Satoshi, Amatya Vishwa Jeet, Tominaga Atsushi, Sugiyama Kazuhiko, Kurisu Kaoru
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.
Childs Nerv Syst. 2016 May;32(5):901-4. doi: 10.1007/s00381-015-2949-2. Epub 2015 Nov 2.
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.
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).
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.
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。
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）。
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。
