Ober K P, Alexander E, Challa V R, Ferree C, Elster A
Department of Medicine, Wake Forest University Medical Center, Winston-Salem, North Carolina.
Neurosurgery. 1989 Jan;24(1):93-5. doi: 10.1227/00006123-198901000-00015.
An 18-year-old woman presented with visual disturbance and endocrine dysfunction (diabetes insipidus, delayed puberty, hypothyroidism, hypoadrenalism, and hyperprolactinemia). Computed tomography and enhanced cisternography showed a single hypothalamic mass, which proved at biopsy to be histiocytosis X. Further studies showed the disease to be restricted to the hypothalamus. The patient was treated with hormonal replacement therapy, but her visual acuity continued to worsen. Visual acuity improved after low-dose irradiation of the pituitary fossa and hypothalamus, which also led to disappearance of the mass. This case shows that localized hypothalamic histiocytosis X can be diagnosed and treated successfully before other systems become involved.
一名18岁女性出现视力障碍和内分泌功能障碍（尿崩症、青春期延迟、甲状腺功能减退、肾上腺皮质功能减退和高催乳素血症）。计算机断层扫描和强化脑池造影显示下丘脑有一个单一肿块，活检证实为组织细胞增多症X。进一步检查显示该疾病仅限于下丘脑。患者接受了激素替代治疗，但其视力持续恶化。垂体窝和下丘脑低剂量放疗后视力改善，肿块也消失。该病例表明，局限性下丘脑组织细胞增多症X在其他系统受累之前可以成功诊断和治疗。
