Surgery for Cushing's syndrome: an historical review and recent ten-year experience.
BACKGROUND
Cushing's syndrome (CS), due to multiple etiologies, is a disorder associated with the ravages of cortisol excess. The purpose of this review article is to provide a historical synopsis of surgery for CS, review a recent 10-year period of operative management at a tertiary care facility, and to outline a practical approach to diagnosis and management.


METHODS
From 1996 to 2005, 298 patients underwent 322 operative procedures for CS at Mayo Clinic, Rochester, Minnesota. A retrospective chart review was carried out. Data was gathered regarding demographics, preoperative assessment, procedures performed, and outcomes. Data are presented as counts and percentages. Five-year survival rates were calculated where applicable by the Kaplan-Meier method. Statistical analysis was carried out with SAS, version 9 (SAS Institute, Inc., Cary, NC).


RESULTS
Two-hundred thirty-one patients (78%) had ACTH-dependent CS and 67 patients (22%) had ACTH-independent CS. One-hundred ninety-six patients (66%) had pituitary-dependent CS and 35 patients (12%) had ectopic ACTH syndrome. Fifty-four patients (18%) had cortisol-secreting adenomas, 10 patients (3%) had cortisol-producing adrenocortical carcinomas, and 1% had other causes. Cure rates for first time pituitary operations (transsphenoidal, sublabial, and endonasal) were 80% and 55% for reoperations. Most benign adrenal processes could be managed laparoscopically. Five-year survival rates (all causes) were 90%, 51%, and 23% for adrenocortical adenomas, ectopic ACTH syndrome, and adrenocortical carcinomas, respectively.


CONCLUSIONS
Surgery for CS is highly successful for pituitary-dependent CS and most ACTH-independent adrenal causes. Bilateral total adrenalectomy can also provide effective palliation from the ravages of hypercortisolism in patients with ectopic ACTH syndrome and for those who have failed transsphenoidal surgery. Unfortunately, to date, adrenocortical carcinomas are rarely cured. Future successes with this disease will likely depend on a better understanding of tumor biology, more effective adjuvant therapies and earlier detection. Clearly, IPSS, advances in cross-sectional imaging, along with developments in transsphenoidal and laparoscopic surgery, have had the greatest impact on today's management of the complex patient with CS.
Porterfield
John R
JR
Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street, S.W, Rochester, MN 55905, USA.
Thompson
Geoffrey B
GB
Young
William F
WF
Chow
John T
JT
Fryrear
Raymond S
RS
van Heerden
Jon A
JA
Farley
David R
DR
Atkinson
John L D
JL
Meyer
Fredric B
FB
Abboud
Charles F
CF
Nippoldt
Todd B
TB
Natt
Neena
N
Erickson
Dana
D
Vella
Adrian
A
Carpenter
Paul C
PC
Richards
Melanie
M
Carney
J Aidan
JA
Larson
Dirk
D
Schleck
Cathy
C
Churchward
Marilyn
M
Grant
Clive S
CS
D015331:Cohort Studies; D003131:Combined Modality Therapy; D003399:Craniotomy; D003480:Cushing Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D004724:Endoscopy; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome