Deficits in the management of patients with adrenocortical carcinoma in Germany.
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.


CONCLUSIONS
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.
Medizinische Klinik und Poliklinik I, Schwerpunkt Endokrinologie und Diabetologie, Universitätsklinikum Würzburg, Germany.
Sarah
S
Johanssen
Stefanie
S
Hahner
Wolfgang
W
Saeger
Marcus
M
Quinkler
Felix
F
Beuschlein
Henning
H
Dralle
Michaela
M
Haaf
Matthias
M
Kroiss
Christian
C
Jurowich
Peter
P
Langer
Wolfgang
W
Oelkers
Martin
M
Spahn
Holger S
HS
Willenberg
Uwe
U
Mäder
Bruno
B
Allolio
Martin
M
Fassnacht
D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003695:Delivery of Health Care / Q000706:statistics & numerical data; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D011300:Preoperative Care; D015995:Prevalence; D011361:Professional Competence / Q000706:statistics & numerical data*; D012042:Registries / Q000706:statistics & numerical data; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
Cancer. 2009 Jul 1;115(13):2816-23
19402169
N Engl J Med. 2009 May 28;360(22):2328-39
19474430
Endocr Rev. 2004 Apr;25(2):309-40
15082524
Endocr Relat Cancer. 2008 Dec;15(4):1043-53
18824557
Cancer. 2009 Jan 15;115(2):243-50
19025987
J Clin Oncol. 2002 Feb 15;20(4):941-50
11844815
J Endocrinol Invest. 2004 Apr;27(4):387-99
15233562
Lancet. 2005 Aug 20-26;366(9486):665-75
16112304
World J Surg. 2006 May;30(5):872-8
16680602
Ann Surg Oncol. 1999 Dec;6(8):719-26
10622498
Curr Opin Investig Drugs. 2005 Apr;6(4):386-94
15898346
Cancer. 2008 Dec 1;113(11):3130-6
18973179
NIH Consens State Sci Statements. 2002 Feb 4-6;19(2):1-25
14768652
N Engl J Med. 2007 Jun 7;356(23):2372-80
17554118
J Clin Endocrinol Metab. 2006 Jun;91(6):2027-37
16551738
Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):273-89
19500769
J Clin Endocrinol Metab. 1994 Dec;79(6):1532-9
7989452
Endocr Relat Cancer. 2007 Mar;14(1):13-28
17395972
Cancer. 1993 Dec 1;72(11):3145-55
8242539
World J Surg. 2001 Jul;25(7):891-7
11572030
Endocr Relat Cancer. 2005 Sep;12(3):667-80
16172199
J Clin Endocrinol Metab. 2006 Jul;91(7):2650-5
16670169
Surgery. 1997 Dec;122(6):1212-8
9426440
Am J Med. 1966 Oct;41(4):572-80
5923598
J Clin Endocrinol Metab. 2009 Jun;94(6):1853-78
19494161
Ann Intern Med. 2003 Mar 4;138(5):424-9
12614096
Urologe A. 2008 Feb;47(2):172-81
18030443
Surgery. 1992 Dec;112(6):963-70; discussion 970-1
1455321
J Clin Endocrinol Metab. 2007 Jan;92(1):148-54
17062775
J Clin Endocrinol Metab. 2009 May;94(5):1713-22
19190108
Exp Clin Endocrinol Diabetes. 2006 Feb;114(2):45-51
16570232
Endocr Rev. 1995 Aug;16(4):460-84
8521790
J Clin Endocrinol Metab. 2006 Nov;91(11):4501-4
16895957
Arch Surg. 1991 Apr;126(4):457-61
1706914