Rahman Sara H, Chittibonia Prashant, Quezado Martha, Patronas Nicholas, Stratakis Constantine A, Lodish Maya B
Clinical Center of the National Institutes of Health (NIH) 10 Center Dr, Bethesda, MD 20814.
Frank H. Netter SOM, Quinnipiac University.
J Clin Transl Endocrinol Case Rep. 2016 Dec;2:30-34. doi: 10.1016/j.jecr.2016.09.001.
We report here a pediatric patient whose Cushing's Disease was diagnosed late because of her cyclical presentation, presumably due to subclinical pituitary apoplexy. Starting at age 8, she presented with observable signs of Cushing's but was not clinically assessed for Cushing's Syndrome until the age of 15. Initial tests at age 15 were consistent with Cushing's Disease, however, the patient presented with spontaneous remission of hypercortisolemia just a few short months later. Her cushingoid features never subsided, and at age 17, her MRI showed a partially empty sella; this finding of an empty sella contributed evidence to our suspicion of asymptomatic apoplexy, especially since the patient never reported an episode of acute headache. Pituitary apoplexy in corticotroph adenomas is very uncommon, but even more rare in microadenomas, making this case very unusual. Lost to follow-up, she was not reevaluated for Cushing's Disease until age 25, and her laboratory tests were consistent with an adrenocorticotrophic-dependent pituitary tumor; Pituitary magnetic resonance imaging revealed a 9 mm X 6 mm X 8 mm mass projecting on the superior aspect of pituitary and abutting the wall of the right cavernous sinus. The patient had a transsphenoidal surgery to remove the microadenoma and is planned to undergo radiation therapy. To the best of our knowledge, this is the first report of subclinical apoplexy of a microadenoma in a pediatric patient with Cushing's Disease. It brings to light the importance of long term follow up for pediatric patients presenting with clinical symptoms of Cushing's Syndrome.
我们在此报告一名儿科患者，其库欣病因周期性表现而诊断较晚，推测是由于亚临床垂体卒中所致。该患者8岁开始出现库欣病的明显体征，但直到15岁才接受库欣综合征的临床评估。15岁时的初步检查结果符合库欣病，但短短几个月后患者出现高皮质醇血症自发缓解。她的库欣样特征从未消退，17岁时，其MRI显示部分空蝶鞍；空蝶鞍这一发现为我们怀疑无症状卒中提供了证据，特别是因为患者从未报告过急性头痛发作。促肾上腺皮质激素腺瘤中的垂体卒中非常罕见，而在微腺瘤中更为罕见，使得该病例非常特殊。该患者失访，直到25岁才重新接受库欣病评估，其实验室检查结果符合促肾上腺皮质激素依赖型垂体肿瘤；垂体磁共振成像显示一个9毫米×6毫米×8毫米的肿块，突出于垂体上方并紧邻右侧海绵窦壁。患者接受了经蝶窦手术切除微腺瘤，并计划接受放射治疗。据我们所知，这是首例关于患有库欣病的儿科患者微腺瘤亚临床卒中的报告。它揭示了对出现库欣综合征临床症状的儿科患者进行长期随访的重要性。
