Guelho Daniela, Paiva Isabel, Vieira Alexandra, Carrilho Francisco
Department of Endocrinology, Diabetes and Metabolism of Coimbra Hospital and University Centre,, Coimbra, Portugal.
Department of Endocrinology, Diabetes and Metabolism of Coimbra Hospital and University Centre,, Coimbra, Portugal.
Endocrinol Nutr. 2016 May;63(5):212-9. doi: 10.1016/j.endonu.2015.12.009. Epub 2016 Mar 9.
Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. The clinical experience acquired, even from a small number of cases, has improved understanding of this condition. The purpose of this study is to characterize patients with ACC followed up at a Portuguese reference center over the past 22 years.
Retrospective analysis of clinical records of patients with histopathological diagnosis of ACC followed up from 1992 to 2014.
The study sample consisted of 22 patients, 20 of them female. Eleven patients were in stage II, four in stage III, and five in stage IV; 13 patients had functioning lesions. Adrenalectomy was performed in 20 patients, with complete tumor resection in 90% of the cases. During follow-up, eight patients experienced recurrence of local disease, and 12 distant metastases. Fourteen patients received mitotane, 35.7% (n=5) as adjuvant therapy and 64.3% (n=9) after recurrence; therapeutic plasma mitotane levels were achieved in 70% of patients. Stage III patients who received adjuvant therapy had longer survival time (13.5 vs. 2.5 months). Two patients were given chemotherapy associated to mitotane. Median survival was 11 months (0-257 months); it was slightly longer in younger patients or patients with non-functioning tumors. Six patients are still alive, four of them with no evidence of disease.
Despite the overall poor prognosis, some patients with ACC may have a long survival time. Although complete tumor removal remains the only potentially curative treatment, diagnosis at a younger age, presence of non-functioning tumors, and mitotane treatment also seemed to be associated to longer survival in our patients.
肾上腺皮质癌（ACC）是一种罕见疾病，预后较差。即使从少数病例中获得的临床经验，也增进了对这种疾病的了解。本研究的目的是描述过去22年在葡萄牙一家参考中心接受随访的ACC患者的特征。
对1992年至2014年随访的组织病理学诊断为ACC的患者的临床记录进行回顾性分析。
研究样本包括22例患者，其中20例为女性。11例患者处于II期，4例处于III期，5例处于IV期；13例患者有功能性病变。20例患者接受了肾上腺切除术，90%的病例实现了肿瘤完全切除。在随访期间，8例患者出现局部疾病复发，12例出现远处转移。14例患者接受了米托坦治疗，35.7%（n = 5）作为辅助治疗，64.3%（n = 9）在复发后使用；70%的患者达到了治疗性血浆米托坦水平。接受辅助治疗的III期患者生存时间更长（13.5个月对2.5个月）。2例患者接受了与米托坦联合的化疗。中位生存期为11个月（0 - 257个月）；年轻患者或无功能性肿瘤患者的生存期略长。6例患者仍然存活，其中4例无疾病证据。
尽管总体预后较差，但一些ACC患者可能有较长的生存时间。虽然完全切除肿瘤仍然是唯一可能治愈的治疗方法，但在我们的患者中，年轻时诊断、无功能性肿瘤的存在以及米托坦治疗似乎也与较长的生存期相关。
