Deficits in the management of patients with adrenocortical carcinoma in Germany

Abstract

Background: 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.

Methods: 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).

Results: 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.

Conclusion: 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.

Figures

Figure 1

Preoperative hormonal work-up in patients with adrenocortical carcinoma in Germany (1998–2009, n = 352, 16–86 years) Definitions: Complete preoperative hormonal work-up: at least 1 test to diagnose hypercortisolism + at least 1 sexual hormone/steroid precursor test + exclusion of pheochromocytoma; Incomplete preoperative hormonal work-up: all other combinations; Missing preoperative hormonal work-up: no endocrine testing

Figure 2

Frequency of imaging procedures used at the time of the initial diagnosis in patients with adrenocortical carcinoma in Germany (1998–2009, n = 370, 16–86 years)

Figure 3

Tumor stage–adjusted survival (Cox regression analysis) by resection status in patients with adrenocortical carcinoma in Germany (1998–2009, n = 180); patients in stage IV and patients with damage to the tumor capsule were excluded. Patients with R2 resection were not analyzed because of the low case numbers (n = 6). R0 = microscopically complete resection (n = 149) R1 = microscopically verifiable tumor residue (n = 16); hazard ratio for death compared to R0 group 3.3; (95% confidence interval: 1.8–6.0; p

Figure 4

Time to first postoperative follow-up (imaging) in patients with adrenocortical carcinoma in Germany (1998–2009, n = 350, 17–86 years)