肾上腺皮质癌（ACC）是一种罕见的侵袭性恶性肿瘤，治疗选择有限。尽管最近的进展揭示了这些肿瘤的基因组驱动因素，但仍不清楚哪些基因组特征与复发相关，特别是在 R0 切除后。使用 cBioPortal 识别了癌症基因组图谱中接受肾上腺切除术治疗的肾上腺皮质癌患者的复发数据。回顾性分析临床病理变量、基因组学、治疗模式和结果。在 92 名肾上腺皮质癌患者中，84 名有复发数据，其中 52% 经历肿瘤复发。复发组和非复发组之间的年龄和性别没有显着差异。非复发患者的总生存期显着延长（54 个月 vs 35 个月，P = .0036）。两组的辅助放射治疗情况相似（25.0% vs 16.2%，P = .4164）。包膜或静脉侵犯或肿瘤中值大小没有差异。 62 名患者进行了 R0 切除，40.3% (n = 25/62) 复发。该队列中的多变量逻辑回归在控制血管侵犯、静脉侵犯和包膜侵犯时显示，WNT（比值比 4.43 [1.09-18.0]，P = 0.034）、PI3K（比值比 7.80 [1.33-45.65] ，P = .023）和细胞周期（比值比 6.81 [1.43-32.30]，P = .016）途径与复发显着相关。复发的中位时间为 7.9 个月；早期复发（<7.9 个月）与 MYC 通路改变相关（40.9% vs 9.1%，P = .0339）。这项研究确定了与肾上腺皮质癌复发相关的 PI3K、WNT 和细胞周期通路中的基因组特征，包括那些接受R0切除术的人。有必要对这些特征作为预测辅助治疗或靶向治疗的预后工具的效用进行调查。版权所有 © 2024 Elsevier Inc. 保留所有权利。

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited treatment options. Although there have been recent advancements revealing genomic drivers of these tumors, it remains unclear which genomic signatures are associated with recurrence, particularly following R0 resection.Adrenocortical carcinoma patients treated with adrenalectomy in the Cancer Genome Atlas with recurrence data were identified using cBioPortal. Clinicopathologic variables, genomics, treatment patterns, and outcomes were retrospectively analyzed.Among 92 adrenocortical carcinoma patients, 84 had recurrence data, with 52% experiencing tumor recurrence. Age and sex were not significantly different between recurrent and nonrecurrent groups. Nonrecurrent patients had a significantly longer overall survival (54 months vs 35 months, P = .0036). Adjuvant radiation was administered similarly in both groups (25.0% vs 16.2%, P = .4164). There were no differences in capsular or venous invasion or median tumor size. Sixty-two patients had R0 resection and 40.3% (n = 25/62) recurred. Multivariate logistic regression in this cohort, when controlling for vascular invasion, venous invasion, and capsular invasion, revealed that the WNT (odds ratio 4.43 [1.09-18.0], P = .034), PI3K (odds ratio 7.80 [1.33-45.65], P = .023), and cell cycle (odds ratio 6.81 [1.43-32.30], P = .016) pathways were significantly associated with recurrence. Median time to recurrence was 7.9 months; early recurrence (<7.9 months) was associated with MYC pathway alterations (40.9% vs 9.1%, P = .0339).This study identified genomic signatures in the PI3K, WNT, and cell cycle pathways associated with adrenocortical carcinoma recurrence, including in those who underwent R0 resection. Investigations regarding the utility of these signatures as a prognostic tool to dictate adjuvant therapies or targeted treatment are warranted.Copyright © 2024 Elsevier Inc. All rights reserved.