Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Front Endocrinol (Lausanne). 2023 Mar 22;14:1138114. doi: 10.3389/fendo.2023.1138114. eCollection 2023.
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.
成人单纯雄激素分泌性肾上腺肿瘤（PASAT）极为罕见，其特征在很大程度上尚未明确。
报告了一例罕见的成人双侧 PASAT 病例，并对成人 PASAT 进行了系统文献回顾，以总结 PASAT 的特征。
本综述共纳入 48 项研究，包括 40 例病例报告和 8 篇文章。对 42 例患者（包括当前病例和 40 例病例报告中的 41 例）数据分析表明，患者平均年龄为 40.48±15.80 岁（18-76 岁）。成人 PASAT 的发病高峰在 21-30 岁，而恶性 PASAT 的发病高峰在 41-50 岁。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）是最常见的升高雄激素（36/42，85.71%），32 例检测患者中有 26 例表现出脱氢表雄酮硫酸酯（DS）水平升高。在恶性肿瘤病例中，疾病持续时间明显缩短（1.96 年比 4.51 年，P=0.025），肿瘤直径明显增大（8.9 厘米比 4.9 厘米，P=0.011）。此外，恶性肿瘤患者的雄激素水平，即 T/上正常范围限（UNRL）（11.94 比 4.943，P=0.770）和 DS/UNRL（16.5 比 5.28，P=0.625）较高。7 例患者中有 5 例在人绒毛膜促性腺激素（HCG）刺激试验中表现出 DS 或 T 升高。总体而言，42 例患者（包括当前病例）接受了肾上腺手术，11 例恶性患者中有 5 例出现复发、转移或死亡，即使进行辅助或挽救性米托坦化疗也是如此。
成人 PASAT 以女性为主，表现为男性化和月经功能障碍，尤其是多毛症。升高的 T 和 DS 可能有助于成人 PASAT 的诊断，HCG 刺激试验也可能有助于诊断。恶性 PASAT 患者的疾病持续时间较短，肿瘤较大，雄激素水平相对较高。建议对所有局部 PASAT 患者进行手术，由于恶性 PASAT 的恶性程度高、预后差和有效治疗方法有限，应充分考虑其恶性肿瘤的可能性。
