Medizinische Klinik und Poliklinik I, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Germany.
Dtsch Arztebl Int. 2010 Dec;107(50):885-91. doi: 10.3238/arztebl.2010.0885. Epub 2010 Dec 17.
Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Often, the physicians who first treat patients with ACC have no prior experience with the disease. The aim of our study was to evaluate the quality of medical care for patients with ACC in Germany.
Data from the German ACC registry were analyzed with regard to the patients' preoperative diagnostic evaluation, histopathological reporting, and clinical follow-up. The findings were compared with the recommendations of the European Network for the Study of Adrenal Tumors (ENSAT).
Data were analyzed from 387 patients who had been given an initial diagnosis of ACC in the years 1998 to 2009. 21% of them underwent no hormonal evaluation before surgery, and 59% underwent an inadequate hormonal evaluation. This exposed the patients to unnecessary perioperative risks and impaired their follow-up. 48% did not undergo CT scanning of the chest, even though the lungs are the most frequent site of metastases of ACC. For 13% of the patients, the diagnosis of ACC was later revised by a reference pathologist. For 11% of the patients, the histopathology report contained no information about resection status, even though this is an important determinant of further treatment and prognosis. Optimal management requires re-staging at three-month intervals, yet some patients underwent re-staging only after a longer delay, or not at all.
We have identified significant deficits in the care of patients with ACC in Germany. We suspect that the situation is similar for other rare diseases. The prerequisite to better care is close and early cooperation of the treating physicians with specialized centers.
肾上腺皮质癌（ACC）是一种罕见的肿瘤，预后不良。通常，首次治疗 ACC 患者的医生以前没有治疗这种疾病的经验。我们的研究目的是评估德国 ACC 患者的医疗质量。
对德国 ACC 登记处的数据进行了分析，涉及患者的术前诊断评估、组织病理学报告和临床随访。结果与欧洲肾上腺肿瘤研究网络（ENSAT）的建议进行了比较。
对 1998 年至 2009 年间初诊为 ACC 的 387 例患者进行了数据分析。其中 21%的患者在手术前未进行激素评估，59%的患者进行了不充分的激素评估。这使患者面临不必要的围手术期风险，并影响了他们的随访。48%的患者未进行胸部 CT 扫描，尽管肺部是 ACC 最常见的转移部位。对于 13%的患者，参考病理学家后来修改了 ACC 的诊断。对于 11%的患者，组织病理学报告未提供关于切除状态的信息，尽管这是进一步治疗和预后的重要决定因素。最佳治疗需要每三个月进行重新分期，但有些患者的重新分期延迟较长，或根本没有进行。
我们已经确定了德国 ACC 患者护理方面存在明显缺陷。我们怀疑其他罕见疾病的情况也类似。更好的护理的前提是治疗医生与专门中心密切及早合作。
