Surgical management of Cushing's syndrome

Cushing's syndrome represents a constellation of symptoms of various origins. In most patients, detailed endocrinologic and radiologic testing will differentiate between Cushing's disease, adrenal adenoma, adrenal carcinoma, primary bilateral nodular hyperplasia, and ectopic corticotropin-producing tumors. Although adrenal surgery affords rapid and reliable remission in patients with Cushing's syndrome, it is associated with significant morbidity and mortality. Complications can be minimized by careful perioperative preparation. The indications for adrenal surgery for Cushing's disease have been altered radically by the success and low morbidity of transsphenoidal surgery. Total adrenalectomy is indicated in patients with bilateral nodular hyperplasia and should be considered for adults who have failed selective pituitary adenectomy or hypophysectomy and in whom ectopic corticotropin secretion has been unequivocally ruled out. At the Lahey Clinic, total adrenalectomy is performed through an anterior abdominal incision. Anterior approaches are especially indicated in those patients who require abdominal exploration for other intra-abdominal pathologic conditions that require surgery. Total adrenalectomy is indicated in the very rare patient who has Cushing's syndrome caused by ectopic corticotropin production when the patient is severely ill, a primary tumor is not found, and medical therapy fails or is poorly tolerated. Small adrenal tumors are best approached through a flank incision. Larger potentially malignant tumors should be approached through a thoracoabdominal incision.