Chauhan Aditya, Ahmed Ammar, Avula Sreekant, Rabe Kimmie, Estrada Allison
Department of Medicine, Division of Endocrinology, Diabetes, and Metabolism, University of Minnesota School of Medicine, Minneapolis, USA.
Department of Pathology and Laboratory Medicine, Hennepin County Medical Center, Minneapolis, USA.
Cureus. 2024 Jul 16;16(7):e64652. doi: 10.7759/cureus.64652. eCollection 2024 Jul.
Langerhans cell histiocytosis (LCH) is a rare disorder involving an abnormal clonal proliferation of precursor cells of the mononuclear phagocytic system. The hypothalamic-pituitary axis is commonly affected by central nervous system (CNS) involvement, with central diabetes insipidus being the most common endocrine abnormality observed. We report the case of a 55-year-old female presenting with vision changes and found to have a hypothalamic mass that was responsive to high-dose steroids. After an initial diagnostic dilemma, the surgical pathology eventually confirmed the diagnosis of LCH. She is being treated with hormone supplementation for panhypopituitarism and intensity-modulated radiation therapy (IMRT) for the LCH. Our case highlights that LCH can present as isolated hypothalamic-pituitary involvement. Early diagnosis is critical to prevent extensive progression of the disease, ultimately leading to permanent physical and endocrine abnormalities. More studies are required to develop specific guidelines and approaches for patients with isolated hypothalamic-pituitary involvement due to LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的疾病，涉及单核吞噬系统前体细胞的异常克隆增殖。下丘脑-垂体轴通常会受到中枢神经系统（CNS）受累的影响，中枢性尿崩症是观察到的最常见的内分泌异常。我们报告了一例55岁女性病例，该患者出现视力变化，发现有一个对大剂量类固醇有反应的下丘脑肿块。经过最初的诊断困境后，手术病理最终确诊为LCH。她正在接受全垂体功能减退的激素替代治疗以及LCH的调强放射治疗（IMRT）。我们的病例强调LCH可表现为孤立的下丘脑-垂体受累。早期诊断对于预防疾病的广泛进展至关重要，最终可导致永久性的身体和内分泌异常。由于LCH导致孤立的下丘脑-垂体受累的患者需要更多研究来制定具体的指南和治疗方法。
