纳尔逊综合征是一种罕见的疾病,以双侧肾上腺切除术后促肾上腺皮质激素(ACTH)分泌垂体大腺瘤引起的临床特征为特征。纳尔逊综合征的常见症状包括体重增加,视力问题,皮肤色素沉着过度,在许多其他人中。在这个案例报告中,本研究对一名58岁的亚裔女性进行了调查,该女性尽管没有接受双侧肾上腺切除术,但仍表现出类似于Nelson综合征的临床特征.该患者过去有分泌ACTH的垂体大腺瘤病史,在放射治疗的同时进行了经蝶窦切除术。一年后,她表现出严重的面部和颈部色素沉着过度。根据获得的实验室结果,患者最初表现出较高的ACTH水平和较低的正常AM皮质醇水平,是部分肾上腺功能不全的征兆.做了脑部核磁共振,证实海绵窦内肿瘤组织稳定残留。结果表明,肾上腺是色素沉着过度的原因,患者被诊断为原发性肾上腺功能不全。为了使她的ACTH水平和正常的低AM皮质醇达到适当的范围,她接受了低剂量氢化可的松治疗,并接受了5年的监测。在这段时间里,她的色素沉着明显改善,最终完全解决,她的ACTH水平降低了,表明氢化可的松是使ACTH水平正常化的适当治疗方法。在这种情况下,确定无反应的肾上腺导致高ACTH水平,这导致了纳尔逊综合征的非典型病例和色素沉着过度的身体症状。
Nelson syndrome is a rare disorder, characterized by clinical features arising from an adrenocorticotropic hormone (ACTH)-secreting pituitary macroadenoma after bilateral adrenalectomy. Common symptoms of Nelson syndrome include weight gain, vision problems, and skin hyperpigmentation, among many others. In this case report, a 58-year-old Asian female who displayed clinical features akin to Nelson syndrome despite not undergoing bilateral adrenalectomy is investigated. The patient has a past history of an ACTH-secreting pituitary macroadenoma, for which a transsphenoidal resection was performed along with radiation therapy. A year following this, she displayed severe facial and neck hyperpigmentation. According to the laboratory results obtained, the patient displayed initial high ACTH levels and low-normal AM cortisol levels, which are signs of partial adrenal insufficiency. A brain MRI was performed, which confirmed stable residual tumor tissue in the cavernous sinus. The results pointed to the adrenal glands as the cause of the hyperpigmentation, and the patient was diagnosed with primary adrenal insufficiency. To bring her ACTH levels and low-normal AM cortisol into the proper range, she was given low-dose hydrocortisone and monitored for five years. Over this time period, her hyperpigmentation improved significantly and eventually resolved entirely, and her ACTH levels were lowered, indicating that hydrocortisone was the appropriate treatment for normalizing ACTH levels. In this case, it was determined that unresponsive adrenal glands lead to high ACTH levels, which resulted in an atypical case of Nelson syndrome and the physical symptom of hyperpigmentation.