Hiéronimus S, Hadjali Y, Fredenrich A, Paquis P, Chanalet S, Grimaud A, Michiels J, Fenichel P
Department of Endocrinology, Hôpital de l'Archet 1, BP 3079, 06202 Nice Cedex 3 France.
Ann Endocrinol (Paris). 2000 Dec;61(6):512-516.
Four cases of hypothalamic-pituitary Langerhans cell histiocytosis (LCH) are reported, highlighting the expanding spectrum of clinical and magnetic resonance imaging (MRI) features in adults. The diagnostic challenge of hypothalamic-pituitary LCH is emphasized in cases revealed as supra-sellar tumors with panhypopituitarism or as isolated central diabetes insipidus. Diagnosis is confirmed by histological examination showing infiltration with CD1a positive histiocytes. General guidelines for diagnosis procedure are drawn out, including the neurosurgical biopsy in particular cases.
本文报告了4例下丘脑-垂体朗格汉斯细胞组织细胞增多症（LCH），强调了成人LCH临床和磁共振成像（MRI）特征范围的不断扩大。对于表现为伴有全垂体功能减退的鞍上肿瘤或孤立性中枢性尿崩症的病例，强调了下丘脑-垂体LCH的诊断挑战。通过组织学检查显示CD1a阳性组织细胞浸润来确诊。制定了诊断程序的一般指南，尤其包括特殊情况下的神经外科活检。
