Churchward, Marilyn

AbstractBackgroundCushing’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 MethodsFrom 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).ResultsTwo‐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.ConclusionsSurgery 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.

AbstractTo evaluate long‐term survival of patients with gastrointestinal carcinoid tumors and to assess factors that may influence prognosis, 154 patients (49% females, 51% males), median age 62 years (range 12–84 years) treated at our institution during 1972–1982 have been followed long term. Tumor location included the foregut (7%), midgut (62%), and hindgut (30%). Ninety‐five percent of the patients underwent surgical or endoscopic excision of the primary tumor, with overall operative mortality and postoperative morbidity rates of 2.6% and 11%, respectively. At follow‐up, 60 patients (39%) were alive (median follow‐up 18 years; range 1–26 years). The main causes of death included carcinoid tumor burden (32%), unrelated causes (45%), other malignancy (19%), and unknown causes (4%). Observed overall 5‐ and 10‐year survivals were 69% and 53%, respectively. Survival was not related to gender or symptoms at presentation. However, age, embryologic origin, tumor size, depth of invasion, nodal status, and stage of disease proved to be of statistical significance (log‐rank). In a multivariate Cox' model, only older age (> 62 years) [P =0.001, odds ratio (OR) = 3.4) and embryologic origin (midgut versus foregut) (P = 0.045, OR = 0.45) provided independent prognostic power when death from any cause was taken as the end‐point. This study confirms that patient's age and the site of the primary tumor have prognostic significance. Carcinoid tumors are neuroendocrine tumors with a relatively good prognosis, and long‐term survival is possible despite advanced stages of disease.