Arnon Johnathan, Grozinsky-Glasberg Simona, Oleinikov Kira, Gross David J, Salmon Asher, Meirovitz Amichay, Maimon Ofra
Department of Oncology, Sharett institute for Oncology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Israel.
Neuroendocrine Unit, ENETS Center of Excellence, Department of Endocrinology and Metabolism, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Israel.
J Endocr Soc. 2022 Jul 26;6(9):bvac112. doi: 10.1210/jendso/bvac112. eCollection 2022 Sep 1.
Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis for both locally advanced and metastatic disease. Standard treatment with combination etoposide-doxorubicin-cisplatin-mitotane (EDP-M) is highly toxic and some patients benefit from mitotane monotherapy. However, identification of these patients remains challenging.
We present a summary of the Israeli national referral center's 20 years of experience in treating advanced ACC, with the aim of identifying prognostic factors and assisting in treatment decision making.
We conducted a retrospective multivariate analysis of patients treated for metastatic or locally advanced ACC at Hadassah Medical Center between 2000 and 2020 to determine clinical, pathological, and treatment factors correlated with overall survival (OS).
In our cohort of 37 patients, a combination of modified European Network for the study of Adrenal Tumors (mENSAT) staging with either grade and R status, or age and symptoms was validated to stratify prognosis ( = .01 and = .03, respectively). Patients who underwent R0 resection followed by radiotherapy or metastasectomy for oligometastatic disease had longer OS than patients with residual disease: median OS of 55 months vs 14 months, respectively, hazard ratio 3.1 (CI 1.4-6.7, = .005). Patients treated with mitotane monotherapy had a significantly better prognosis, yet this result was attenuated in a multivariate analysis controlling for mENSAT and R status. Of patients treated with EDP-M, 41.4% experienced grade 3 or higher adverse events.
Patients with advanced ACC achieving R0 status have a better prognosis and might benefit from mitotane monotherapy.
肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤，局部晚期和转移性疾病的预后均较差。依托泊苷-阿霉素-顺铂-米托坦联合（EDP-M）的标准治疗毒性很大，一些患者从米托坦单药治疗中获益。然而，识别这些患者仍然具有挑战性。
我们总结了以色列国家转诊中心20年来治疗晚期ACC的经验，旨在确定预后因素并协助治疗决策。
我们对2000年至2020年在哈达萨医疗中心接受转移性或局部晚期ACC治疗的患者进行了回顾性多变量分析，以确定与总生存期（OS）相关的临床、病理和治疗因素。
在我们的37例患者队列中，改良的欧洲肾上腺肿瘤研究网络（mENSAT）分期与分级和R状态或年龄和症状的组合被证实可用于分层预后（分别为=0.01和=0.03）。接受R0切除，随后对寡转移疾病进行放疗或转移灶切除术的患者比有残留疾病的患者OS更长：中位OS分别为55个月和14个月，风险比为3.1（CI 1.4-6.7，=0.005）。接受米托坦单药治疗的患者预后明显更好，但在控制mENSAT和R状态的多变量分析中，这一结果有所减弱。接受EDP-M治疗的患者中，41.4%发生3级或更高等级的不良事件。
达到R0状态的晚期ACC患者预后较好，可能从米托坦单药治疗中获益。
