TY  - JOUR
A1  - Baur, Johannes
A1  - Büntemeyer, Tjark-Ole
A1  - Megerle, Felix
A1  - Deutschbein, Timo
A1  - Spitzweg, Christine
A1  - Quinkler, Marcus
A1  - Nawroth, Peter
A1  - Kroiss, Matthias
A1  - Germer, Christoph-Thomas
A1  - Fassnacht, Martin
A1  - Steger, Ulrich
T1  - Outcome after resection of Adrenocortical Carcinoma liver metastases: a retrospective study
JF  - BMC Cancer
N2  - Background:
Metastatic Adrenocortical Carcinoma (ACC) is a rare malignancy with a poor 5-year-survival rate (<15%). A surgical approach is recommended in selected patients if complete resection of distant metastasis can be achieved. To date there are only limited data on the outcome after surgical resection of hepatic metastases of ACC.

Methods:
A retrospective analysis of the German Adrenocortical Carcinoma Registry was conducted. Patients with liver metastases of ACC but without extrahepatic metastases or incomplete tumour resection were included.

Results:
Seventy-seven patients fulfilled these criteria. Forty-three patients underwent resection of liver metastases of ACC. Complete tumour resection (R0) could be achieved in 30 (69.8%). Median overall survival after liver resection was 76.1 months in comparison to 10.1 months in the 34 remaining patients with unresected liver metastases (p < 0.001). However, disease free survival after liver resection was only 9.1 months. Neither resection status (R0/R1) nor extent of liver resection were significant predictive factors for overall survival. Patients with a time interval to the first metastasis/recurrence (TTFR) of greater than 12 months or solitary liver metastases showed significantly prolonged survival.

Conclusions:
Liver resection in the case of ACC liver metastases can achieve long term survival with a median overall survival of more than 5 years, but disease free survival is short despite metastasectomy. Time to recurrence and single versus multiple metastases are predictive factors for the outcome.
KW  - Adrenocortical Carcinoma
KW  - liver resection
KW  - retrospective study
KW  - prognosis
KW  - survival analysis
Y1  - 2017
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-159409
VL  - 17
IS  - 522
ER  - 
TY  - JOUR
A1  - Johanssen, Sarah
A1  - Hahner, Stefanie
A1  - Saeger, Wolfgang
A1  - Quinkler, Marcus
A1  - Beuschlein, Felix
A1  - Dralle, Henning
A1  - Haaf, Michaela
A1  - Kroiss, Matthias
A1  - Jurowich, Christian
A1  - Langer, Peter
A1  - Oelkers, Wolfgang
A1  - Spahn, Martin
A1  - Willenberg, Holger S.
A1  - Maeder, Uwe
A1  - Allolio, Bruno
A1  - Fassnacht, Martin
T1  - Deficits in the Management of Patients With Adrenocortical Carcinoma in Germany
N2  - 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 followup. 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.
Y1  - 2010
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-85897
ER  -