■原发性垂体炎是指与其他次要原因无关的垂体的孤立炎症。在其组织病理学亚型中,黄瘤是最罕见的。
■我们描述了一位22岁的女性,患有黄瘤性垂体炎(XH),其8年的临床进展以及泼尼松龙和硫唑嘌呤的治疗效果。我们的患者在14岁时因严重身材矮小和青春期延迟而首次转诊。
■调查显示多种垂体缺陷。磁共振成像显示垂体肿块,因此进行了部分切除。由于肿块的位置,完全切除是不可行的。组织病理学分析与XH一致。肿瘤二次锻炼的结果,感染,自身免疫,炎症性疾病呈阴性。手术后,观察到肿块逐渐增大。给予两个疗程的泼尼松龙,质量显着减少。添加硫唑嘌呤是由于泼尼松龙逐渐减少时的不持续作用以及继续使用类固醇毒性的担忧。使用硫唑嘌呤6个月后,没有发现质量进一步增加。
■糖皮质激素和免疫疗法是XH的治疗选择;然而,需要更多的病例来更好地了解其发病机制和临床进展。
UNASSIGNED: Primary hypophysitis refers to the isolated inflammation of the pituitary gland not associated with other secondary causes. Among its histopathologic subtypes, xanthomatous is the rarest.
UNASSIGNED: We describe a 22-year-old woman with xanthomatous hypophysitis (XH), its clinical progression over 8 years as well as the treatment effects of prednisolone and azathioprine. Our patient was first referred for severe short stature and delayed puberty at the age of 14 years.
UNASSIGNED: Investigations revealed multiple pituitary deficiencies. Magnetic resonance imaging showed a pituitary mass whereby a partial resection was performed. A full resection was not feasible due to the location of the mass. The histopathologic analysis of the tissue was consistent with XH. The results of secondary workout for neoplasm, infection, autoimmune, and inflammatory disorders were negative. After surgery, a progressive enlargement of the mass was observed. Two courses of prednisolone were administered with a significant reduction in the mass size. Azathioprine was added due to the unsustained effects of prednisolone when tapered off and the concern of steroid toxicity with continued use. No further increase in the mass size was noted after 6 months on azathioprine.
UNASSIGNED: Glucocorticoid and immunotherapy are treatment options for XH; however, more cases are needed to better understand its pathogenesis and clinical progression.