Ragnarsson Oskar, Johannsson Gudmundur, Geterud Kjell, Lodding Par, Dahlqvist Per
Department of Endocrinology, Diabetes and Metabolism, Sahlgrenska University Hospital, Gothenburg, Sweden.
BMJ Case Rep. 2013 Aug 13;2013:bcr2013010395. doi: 10.1136/bcr-2013-010395.
Androgen deprivation is a cornerstone in prostate cancer management. We present a 69-year-old man, with a poorly differentiated prostate cancer with skeletal and lymph node metastases. After medical and subsequent surgical castration serum testosterone concentrations remained inappropriately high (4.9 and 4.5 nmol/L; castration range < 0.5). For cancer staging a CT was performed which showed bilateral adrenal enlargement. Endocrine workup revealed elevated levels of adrenal androgens and adrenal precursors. Mutation analysis confirmed a non-classical 21-hydroxylase deficiency, that is, a mild form of congenital adrenal hyperplasia (CAH). To suppress adrenocorticotrophic hormone and the excess adrenal androgen secretion, treatment with hydrocortisone and prednisolone was started with success. Inadequate testosterone suppression after castration due to previously undiagnosed CAH has not previously been reported. Considering the estimated prevalence of 1% in selected populations, non-classical CAH should be considered when testosterone is not adequately suppressed after castration in men with prostate cancer.
雄激素剥夺是前列腺癌治疗的基石。我们报告一名69岁男性，患有低分化前列腺癌并伴有骨骼和淋巴结转移。在药物去势及随后的手术去势后，血清睾酮浓度仍异常升高（分别为4.9和4.5 nmol/L；去势范围<0.5）。为进行癌症分期，行CT检查显示双侧肾上腺增大。内分泌检查发现肾上腺雄激素和肾上腺前体水平升高。突变分析证实为非经典型21-羟化酶缺乏，即先天性肾上腺皮质增生症（CAH）的一种轻度形式。为抑制促肾上腺皮质激素及过量肾上腺雄激素分泌，开始使用氢化可的松和泼尼松龙治疗并取得成功。此前尚未报道过因既往未诊断的CAH导致去势后睾酮抑制不足的情况。考虑到特定人群中估计患病率为1%，对于前列腺癌男性患者去势后睾酮未得到充分抑制时，应考虑非经典型CAH。
