Adjuvant Therapies and Patient and Tumor Characteristics Associated With Survival of Adult Patients With Adrenocortical Carcinoma
Adjuvant Therapies and Patient and Tumor Characteristics Associated With Survival of Adult Patients With Adrenocortical Carcinoma
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DOI：
10.1210/jc.2013-2856
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发表时间：
2014-02-01
影响因子：
5.8
通讯作者：
Hammer, Gary D.
中科院分区：
文献类型：
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作者：
Else, Tobias;Williams, Andrew R.;Hammer, Gary D.
背景：肾上腺皮质癌是一种罕见的恶性内分泌肿瘤。关于结果和预后因素的研究依赖于相当小的研究。在这里，我们总结了经验的患者诊断肾上腺皮质癌从一个大三级转诊中心。目的：本研究的目的是确定影响肾上腺皮质癌患者预后的因素，并评估辅助治疗策略。设计：采用回顾性单中心研究收集患者资料。在Cox回归模型（多变量和单变量）中分析流行病学、患者和肿瘤特征对总生存率和无复发生存率的预后因素。结果：确诊成人肾上腺皮质癌391例。中位总生存期为35.2个月。皮质醇生成[危险比（HR） 1.4, HR 1.5]，肿瘤分期（HR 3期为2.1和2.1，HR 4期为4.8）和肿瘤分级（HR 2.4和2.0）被确定为负面预后因素（HR为死亡，HR为复发）。米托坦治疗可增加无复发生存期，辅助放射治疗可进一步显著改善这一效果，但不影响总生存期。开腹肾上腺切除术患者的总生存率提高。结论：本研究增加了不良危险因素（皮质醇分泌、高肿瘤分期和高肿瘤分级）的证据，并建议以下治疗方法：肾上腺皮质癌患者应行开放性肾上腺切除术。辅助治疗，特别是米托坦治疗联合放疗，应考虑延缓肿瘤复发。
Context: Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center.Objective: The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies.Design: Patient data were collected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox regression models (multivariable and univariable).Results: Three hundred ninety-one adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production [hazard ratio (HR) 1.4, HR 1.5], tumor stage (HR stage 3 of 2.1 and 2.1, HR stage 4 of 4.8), and tumor grade (HR 2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy but did not impact overall survival. Patients with open adrenalectomy had improved overall survival.Conclusions: This study increases the evidence for adverse risk factors (cortisol production, high tumor stage, and high tumor grade) and suggests the following therapy approach: adrenocortical carcinoma patients should be treated with open adrenalectomy. Adjuvant therapy, particularly mitotane therapy in conjunction with radiation, should be considered to delay tumor recurrence.