Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. Accordingly, in many cases, the attending doctors have no previous experience with the disease. The aim of our study was to evaluate the quality of care in a large number of patients with ACC in Germany. Data from 263 adult patients of the German ACC registry were analyzed with regard to the following parameters: time to diagnosis, hormonal assessment, imaging, histopathological documentation, follow-up. The time from first symptoms to diagnosis varied widely, e.g. in patients with Cushings syndrome from several weeks to 36 months (median 8.5 months). Preoperative diagnostic work up was frequently insufficient: In the majority of patients no (16%) or only incomplete (46%) hormonal investigations were performed, putting the patient at unnecessary risk and impairing future follow-up. Furthermore, in 47% no thoracic CT was performed. Histopathological assessment by the respective local pathologists proved frequently not reliable: during the last two years, the diagnosis of ACC was revised by the reference pathologist of the registry in 7 cases. In addition, resection status was not given in the histopathological report in 15.4% of patients, although this information is of key importance for treatment and prognosis. We detected deficits also in follow-up: due to the high rate of recurrence in ACC, regular restaging every 3 months is recommended. However, in a third of patients a first postoperative follow-up was performed only after more than 6 months. In conclusion, we have identified significant deficits in the care of patients with ACC in Germany. Based on recommendations of the European adrenal network ENSAT only 33% of the patients underwent sufficient hormonal assessment, staging, and postoperative follow-up. Close and early interaction of physicians with specialized centres will be of key importance to improve the care of patients with this rare disease.
03 - 07 May 2008
European Society of Endocrinology