■成人纯分泌雄激素的肾上腺肿瘤(PASATs)极为罕见,它们的特征在很大程度上是未知的。
■报告了一例罕见的成人双侧PASATs,并对成人PASATs进行了系统的文献综述,总结了PASATs的特点。
■总共,48项研究,包括40份病例报告和8篇文章,在这次审查中确定。根据42例患者(包括当前病例和40例病例报告中的41例患者)的数据进行的分析显示,平均年龄为40.48±15.80岁(范围为18-76岁)。成人PASAT的发病率在21-30岁达到高峰,而恶性PASAT在41-50岁时达到顶峰。大多数PASAT患者为女性(40/42,95.23%),多毛症是最常见的症状(37/39,94.87%)。睾酮(T)是最常见的雄激素升高(36/42,85.71%),32例受试患者中有26例出现硫酸脱氢表雄酮(DS)水平升高。在恶性肿瘤病例中,疾病持续时间显著缩短(1.96vs.4.51年,P=0.025),和肿瘤直径显著增加(8.9vs.4.9cm,p=0.011)。此外,雄激素水平,即,T/正常范围上限(UNRL)(11.94vs.4.943,P=0.770)和DS/UNRL(16.5vs.5.28,P=0.625),在恶性肿瘤患者中更高。总的来说,在人绒毛膜促性腺激素(HCG)刺激试验中,7例患者中有5例显示DS或T增加。总的来说,42例患者中有41例(包括当前病例)接受了肾上腺手术,和复发,转移,在11例恶性患者中,有5例报告死亡,即使是辅助或抢救米托坦化疗。
■成人PASAT,这在女性中占主导地位,以男性化和月经功能障碍为特征,尤其是多毛症。T和DS升高可能有助于成人PASAT的诊断,和HCG刺激测试也可能有助于诊断。恶性PASAT患者的病程较短,较大的肿瘤大小和相对较高的雄激素水平。建议对所有当地的PASAT进行手术,由于恶性肿瘤的高风险,应充分考虑PASAT的恶性肿瘤,预后差,有效方法有限。
Adult pure androgen-secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.
A rare
case of adult bilateral PASATs was reported, and a systematic literature review of adult PASATs was conducted to summarize the characteristics of PASATs.
In total, 48 studies, including 40
case reports and 8 articles, were identified in this review. Analysis based on data of 42 patients (including current
case and 41 patients from 40
case reports) showed that average age was 40.48 ± 15.80 years (range of 18-76). The incidence of adult PASAT peaked at 21-30 years old, while that of malignant PASAT peaked at 41-50 years old. Most PASAT patients were female (40/42, 95.23%), and
hirsutism was the most common symptom (37/39, 94.87%). Testosterone (T) was the most commonly elevated androgen (36/42, 85.71%), and 26 of 32 tested patients presented increased dehydroepiandrosterone sulfate (DS) levels. In malignancy cases, disease duration was significantly decreased (1.96 vs. 4.51 years, P=0.025), and tumor diameter was significantly increased (8.9 vs. 4.9 cm, p=0.011). Moreover, the androgen levels, namely, T/upper normal range limit (UNRL) (11.94 vs. 4.943, P=0.770) and DS/UNRL (16.5 vs. 5.28, P=0.625), were higher in patients with malignancy. In total, 5 out of 7 patients showed an increase in DS or T in the human chorionic gonadotropin (HCG) stimulation test. Overall, 41 out of 42 patients (including current case) underwent adrenal surgery, and recurrence, metastasis, or death was reported in 5 out of 11 malignant patients even with adjuvant or rescue mitotane chemotherapy.
Adult PASAT, which is predominant in women, is characterized by virilism and menstrual dysfunction, especially
hirsutism. Elevated T and DS may contribute to the diagnosis of adult PASAT, and HCG stimulation test might also be of help in diagnosis. Patients with malignant PASAT have a shorter disease duration, larger tumor sizes and relatively higher androgen levels. Surgery is recommended for all local PASATs, and Malignancy of PASAT should be fully considered due to the high risk of malignancy, poor prognosis and limited effective approaches.