目前的证据表明，与非分泌性肾上腺皮质癌相比，分泌皮质醇的肾上腺皮质癌的预后较差。然而，其他分泌亚型的影响尚不清楚。美国-澳大利亚-亚洲肾上腺联盟的这项多中心研究包括患有肾上腺皮质癌的成年人（1997-2020）。我们比较了皮质醇分泌型、混合皮质醇/雄激素分泌型、雄激素分泌型和非分泌型肾上腺皮质癌的总生存率和无病生存率。 在 807 名患者（平均年龄 50 岁）中，719 名患者纳入分泌型亚型分析：24.5% 为分泌型肾上腺皮质癌。皮质醇分泌，13% 雄激素分泌，28% 混合皮质醇/雄激素，32.5% 非分泌，2% 为盐皮质激素分泌。整个队列的中位总生存期和无病生存期分别为 60 个月和 9 个月。皮质醇患者的中位总生存期为 36 个月，混合型患者为 30 个月，雄激素分泌型患者为 60 个月，非分泌型肾上腺皮质癌患者为 115 个月，P < .01。皮质醇的中位无病生存期为 7 个月，混合型的中位无病生存期为 8 个月，雄激素型的中位无病生存期为 10 个月，非分泌性肾上腺皮质癌的中位无病生存期为 12 个月，P = .06。在年龄、性别、Ki67%、分泌亚型、分期、切除和辅助治疗的多变量分析中，总生存率较差的预测因素是年龄较大、Ki67%较高、IV期、混合分泌、R1和无辅助治疗，P < .05.在 R0 切除的亚组分析中，总体生存率较差的预测因素包括年龄较大和 Ki67% 较高。 Ki67% ≥40、III 期和皮质醇分泌与较差的无病生存率相关。混合分泌皮质醇/雄激素的肾上腺皮质癌与较差的总生存率相关，而单独分泌皮质醇或雄激素则不然。值得注意的是，在 R0 切除后的患者中，分泌亚型并不影响总生存期。分泌皮质醇的肾上腺皮质癌表现出更差的无病生存率。 Ki67% 仍然是独立于分期的较差总体生存率和无病生存率的有力预测因子。版权所有 © 2023 Elsevier Inc. 保留所有权利。

Current evidence suggests that cortisol secreting adrenocortical carcinoma has worse prognosis compared to non-secreting adrenocortical carcinoma. However, the effect of other secretory subtypes is unknown.This multicenter study within the American-Australian-Asian Adrenal Alliance included adults with adrenocortical carcinoma (1997-2020). We compared overall survival and disease-free survival among cortisol secreting, mixed cortisol/androgen secreting, androgen secreting, and non-secreting adrenocortical carcinoma.Of the 807 patients (mean age 50), 719 included in the secretory subtype analysis: 24.5% were cortisol secreting, 13% androgen secreting, 28% mixed cortisol/androgen, 32.5% non-secreting, and 2% were mineralocorticoid secreting. Median overall survival and disease-free survival for the entire cohort were 60 and 9 months, respectively. Median overall survival was 36 months for cortisol, 30 for mixed, 60 for androgen secreting, and 115 for non-secreting adrenocortical carcinoma, P < .01. Median disease-free survival was 7 months for cortisol, 8 for mixed, 10 for androgen, and 12 for non-secreting adrenocortical carcinoma, P = .06. On multivariable analysis of age, sex, Ki67%, secretory subtype, stage, resection, and adjuvant therapy, predictors of worse overall survival were older age, higher Ki67%, stage IV, mixed secreting, R1, and no adjuvant therapy, P < .05. On subgroup analysis of R0 resection, predictors of worse overall survival included older age and higher Ki67%. Ki67% ≥40, stage III and cortisol secretion were associated with worse disease-free survival.Mixed cortisol/androgen secreting adrenocortical carcinoma was associated with worse overall survival, while cortisol or androgen secreting alone were not. Notably, among patients after R0 resection, secretory subtype did not affect overall survival. Cortisol secreting adrenocortical carcinoma demonstrated worse disease-free survival. Ki67% remained a strong predictor of worse overall survival and disease-free survival independent of stage.Copyright © 2023 Elsevier Inc. All rights reserved.