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Published in: World Journal of Surgery 5/2008

01-05-2008

Surgery for Cushing’s Syndrome: An Historical Review and Recent Ten-year Experience

Authors: John R. Porterfield, Geoffrey B. Thompson, William F. Young Jr., John T. Chow, Raymond S. Fryrear, Jon A. van Heerden, David R. Farley, John L. D. Atkinson, Fredric B. Meyer, Charles F. Abboud, Todd B. Nippoldt, Neena Natt, Dana Erickson, Adrian Vella, Paul C. Carpenter, Melanie Richards, J. Aidan Carney, Dirk Larson, Cathy Schleck, Marilyn Churchward, Clive S. Grant

Published in: World Journal of Surgery | Issue 5/2008

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Abstract

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.

Materials and 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.
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Metadata
Title
Surgery for Cushing’s Syndrome: An Historical Review and Recent Ten-year Experience
Authors
John R. Porterfield
Geoffrey B. Thompson
William F. Young Jr.
John T. Chow
Raymond S. Fryrear
Jon A. van Heerden
David R. Farley
John L. D. Atkinson
Fredric B. Meyer
Charles F. Abboud
Todd B. Nippoldt
Neena Natt
Dana Erickson
Adrian Vella
Paul C. Carpenter
Melanie Richards
J. Aidan Carney
Dirk Larson
Cathy Schleck
Marilyn Churchward
Clive S. Grant
Publication date
01-05-2008
Publisher
Springer-Verlag
Published in
World Journal of Surgery / Issue 5/2008
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI
https://doi.org/10.1007/s00268-007-9387-6

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