Tran Thuy B, Postlewait Lauren M, Maithel Shishir K, Prescott Jason D, Wang Tracy S, Glenn Jason, Phay John E, Keplinger Kara, Fields Ryan C, Jin Linda X, Weber Sharon M, Salem Ahmed, Sicklick Jason K, Gad Shady, Yopp Adam C, Mansour John C, Duh Quan-Yang, Seiser Natalie, Solorzano Carmen C, Kiernan Colleen M, Votanopoulos Konstantinos I, Levine Edward A, Hatzaras Ioannis, Shenoy Rivfka, Pawlik Timothy M, Norton Jeffrey A, Poultsides George A
Department of Surgery, Stanford University School of Medicine, Stanford, California.
Department of Surgery, Emory University, Atlanta, Georgia.
J Surg Oncol. 2016 Dec;114(8):971-976. doi: 10.1002/jso.24439. Epub 2016 Sep 16.
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options beyond surgical resection. The characteristics of actual long-term survivors following surgical resection for ACC have not been previously reported.
Patients who underwent resection for ACC at one of 13 academic institutions participating in the US Adrenocortical Carcinoma Group from 1993 to 2014 were analyzed. Patients were stratified into four groups: early mortality (died within 2 years), late mortality (died within 2-5 years), actual 5-year survivor (survived at least 5 years), and actual 10-year survivor (survived at least 10 years). Patients with less than 5 years of follow-up were excluded.
Among the 180 patients available for analysis, there were 49 actual 5-year survivors (27%) and 12 actual 10-year survivors (7%). Patients who experienced early mortality had higher rates of cortisol-secreting tumors, nodal metastasis, synchronous distant metastasis, and R1 or R2 resections (all P < 0.05). The need for multi-visceral resection, perioperative blood transfusion, and adjuvant therapy correlated with early mortality. However, nodal involvement, distant metastasis, and R1 resection did not preclude patients from becoming actual 10-year survivors. Ten of twelve actual 10-year survivors were women, and of the seven 10-year survivors who experienced disease recurrence, five had undergone repeat surgery to resect the recurrence.
Surgery for ACC can offer a 1 in 4 chance of actual 5-year survival and a 1 in 15 chance of actual 10-year survival. Long-term survival was often achieved with repeat resection for local or distant recurrence, further underscoring the important role of surgery in managing patients with ACC. J. Surg. Oncol. 2016;114:971-976. © 2016 Wiley Periodicals, Inc.
肾上腺皮质癌（ACC）是一种罕见的侵袭性恶性肿瘤，除手术切除外治疗选择有限。此前尚未报道过ACC手术切除后实际长期存活者的特征。
对1993年至2014年期间在美国肾上腺皮质癌研究组参与研究的13家学术机构中接受ACC切除术的患者进行分析。患者被分为四组：早期死亡（2年内死亡）、晚期死亡（2至5年内死亡）、实际5年存活者（存活至少5年）和实际10年存活者（存活至少10年）。随访时间不足5年的患者被排除。
在可供分析的180例患者中，有49例实际5年存活者（27%）和12例实际10年存活者（7%）。早期死亡的患者中，分泌皮质醇肿瘤、淋巴结转移、同时性远处转移以及R1或R2切除的发生率较高（均P<0.05）。多脏器切除、围手术期输血和辅助治疗的需求与早期死亡相关。然而，淋巴结受累、远处转移和R1切除并不妨碍患者成为实际10年存活者。12例实际10年存活者中有10例为女性，在7例经历疾病复发的10年存活者中，有5例接受了再次手术切除复发灶。
ACC手术可提供四分之一的实际5年存活机会和十五分之一的实际10年存活机会。通过对局部或远处复发进行再次切除，常常可实现长期存活，这进一步强调了手术在ACC患者管理中的重要作用。《外科肿瘤学杂志》2016年；114：971 - 976。©2016威利期刊公司
