Daitch J A, Goldfarb D A, Novick A C
Department of Urology, Cleveland Clinic Foundation, Ohio, USA.
J Urol. 1997 Dec;158(6):2051-5; quiz 2275. doi: 10.1016/s0022-5347(01)68151-5.
Cushing's syndrome due to adrenal adenoma or adrenocortical carcinoma is rare. To understand better the clinical and biochemical presentation of this disorder, as well as therapy efficacy and patient survival, we conducted a retrospective review.
Between August 1971 and April 1994, 40 patients presented to our institution with adrenal Cushing's syndrome (27 adenomas and 13 carcinomas). These groups were analyzed with respect to clinical signs and symptoms preoperatively and postoperatively, biochemical analysis, length of postoperative steroid replacement therapy, disease recurrence and patient survival. Followup was obtained by chart review and telephone interviews and averaged 59.6 +/- 66.4 and 47.6 +/- 56.2 months for adenoma and carcinoma patients, respectively.
Women predominated in both groups (26 of 27 adenomas, 11 of 13 carcinomas), and tumors affected the left adrenal gland more frequently (19 of 27 adenomas, 9 of 13 carcinomas). Adenoma patients were younger than carcinoma patients (39.6 +/- 14.4 versus 51.5 +/- 16.6 years, p = 0.026) and presented with smaller tumors (3.3 +/- 1.0 versus 8.6 +/- 4.5 cm., p = 0.001). There was a trend toward increased incidence of glucose intolerance among carcinoma patients but no significant differences in clinical signs or symptoms between adenoma and carcinoma patients could be made. Similarly, while there was no significant difference in biochemical evaluation of adenoma versus carcinoma patients, 24-hour urinary free cortisol and serum lactate dehydrogenase levels tended to be higher among carcinoma patients. In addition 17-ketosteroid and dehydroepiandrosterone sulfate levels were more elevated in carcinoma than in adenoma patients, and several adenoma patients actually had subnormal levels. Among adenoma patients mean length of steroid replacement therapy was 16.8 +/- 9.1 months. However, 7 adenoma patients (25.9%) required greater than 24 months of exogenous steroids, and only 1 of these patients was subsequently weaned off steroid replacement. There were no recurrences among adenoma patients, although there was 1 perioperative death due to hypoglycemia. Ten (76.9%) carcinoma patients had recurrences at a mean followup of 33 months. The 3 and 5-year survival rates were 41.5 and 31.2%, respectively.
While presenting signs and symptoms and hormonal analysis may suggest benign or malignant disease, only tumor size and patient age are reliable preoperative indicators of adrenal adenoma versus adrenocortical carcinoma among patients with adrenal Cushing's syndrome. Surgery is curative for adenoma patients, but lifelong steroid replacement may be required. Survival remains poor among carcinoma patients.
由肾上腺腺瘤或肾上腺皮质癌引起的库欣综合征较为罕见。为了更好地了解这种疾病的临床和生化表现，以及治疗效果和患者生存率，我们进行了一项回顾性研究。
1971年8月至1994年4月期间，40例患者因肾上腺库欣综合征前来我院就诊（27例腺瘤和13例癌）。对这些患者术前和术后的临床体征和症状、生化分析、术后类固醇替代治疗的时长、疾病复发情况和患者生存率进行了分析。通过查阅病历和电话访谈进行随访，腺瘤患者和癌患者的随访平均时长分别为59.6±66.4个月和47.6±56.2个月。
两组患者中女性均占多数（27例腺瘤中有26例，13例癌中有11例），且肿瘤更常累及左侧肾上腺（27例腺瘤中有19例，13例癌中有9例）。腺瘤患者比癌患者年轻（39.6±14.4岁对51.5±16.6岁，p = 0.026），肿瘤也较小（3.3±1.0厘米对8.6±4.5厘米，p = 0.001）。癌患者中糖耐量异常的发生率有上升趋势，但腺瘤患者和癌患者在临床体征或症状方面无显著差异。同样，腺瘤患者和癌患者在生化评估方面无显著差异，但癌患者的24小时尿游离皮质醇和血清乳酸脱氢酶水平往往更高。此外，癌患者的17-酮类固醇和硫酸脱氢表雄酮水平比腺瘤患者升高得更多，一些腺瘤患者实际上水平低于正常。腺瘤患者的类固醇替代治疗平均时长为16.8±9.1个月。然而，7例腺瘤患者（25.9%）需要超过24个月的外源性类固醇治疗，其中只有1例患者随后停用了类固醇替代治疗。腺瘤患者无复发情况，不过有1例患者围手术期因低血糖死亡。10例（76.9%）癌患者在平均33个月的随访中有复发情况。3年和5年生存率分别为41.5%和31.2%。
虽然临床表现和激素分析可能提示良性或恶性疾病，但在肾上腺库欣综合征患者中，只有肿瘤大小和患者年龄是肾上腺腺瘤与肾上腺皮质癌可靠的术前指标。手术对腺瘤患者可治愈，但可能需要终身类固醇替代治疗。癌患者的生存率仍然很低。
