Porterfield, John R.

AbstractBackgroundToxic multinodular goiter (Plummer’s disease) has posed challenges to surgeons, endocrinologists, and radiation oncologists since its description in 1913. A literature review with evidenced‐based methodology has not yet been reported.MethodsA systematic review of the English literature from 1950 to 2007 and report of Mayo Clinic experience since 1950 was undertaken to establish evidence‐based recommendations for management.ResultsSurgery and radioactive iodine (RI) are both supported by level IV evidence in the treatment of solitary toxic nodules and toxic multinodular goiter, and treatment is determined by symptoms and co‐morbidities. No evidence suggests a difference in treatment outcome based on pretreatment clinical or subclinical hyperthyroidism. Level IV evidence supports thyroidectomy over RI for large goiters. When compressive symptoms are present, level IV evidence supports thyroidectomy for maximal symptom relief in patients at moderate risk. Occult malignancies are found in 2–3% of thyroidectomy specimens for Plummer’s disease. Despite technical reports of RI dose considerations, there are no prospective studies validating a dose formula. Ethanol ablation of toxic nodules in patients unfit for surgery is supported by level III evidence. Level V data suggest a cost benefit favoring surgery.ConclusionsTreatment of Plummer’s disease with antithyroid medications, ethanol ablation, RI ablation, or surgery must balance the goals of therapy, durability of cure, relief of symptoms, risk of malignancy, and risk of complications. Between 1950 and 2006, 948 (70%) of 1,356 patients with Plummer’s disease have been treated surgically at Mayo Clinic.

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.