{"id":4422274,"date":"2025-01-11T11:45:26","date_gmt":"2025-01-11T17:45:26","guid":{"rendered":"https:\/\/myendoconsult.com\/learn\/topics\/cushings-disease\/"},"modified":"2025-01-13T06:51:16","modified_gmt":"2025-01-13T12:51:16","slug":"cushings-disease","status":"publish","type":"oen_topic","link":"https:\/\/myendoconsult.com\/learn\/topics\/cushings-disease\/","title":{"rendered":"Cushing&#8217;s Disease"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">CORTICOTROPIN (ACTH)-SECRETING PITUITARY TUMOR<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Overview and Definition<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Excess Cortisol Production<\/strong>: Corticotropin (ACTH)-secreting pituitary adenomas stimulate excess adrenal secretion of cortisol, resulting in the clinical picture of Cushing syndrome.<\/li>\n\n\n\n<li><strong>Typical Pathology<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Usually <strong>benign microadenomas<\/strong> (\u226410 mm).<\/li>\n\n\n\n<li>Occasionally macroadenomas, and very rarely carcinomas.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">SURGICAL TREATMENT<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment of Choice<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Transsphenoidal Selective Adenectomy<\/strong>:\n<ul class=\"wp-block-list\">\n<li>The definitive therapy for ACTH-secreting pituitary adenomas.<\/li>\n\n\n\n<li>Surgical success is defined by <strong>cure of <a href=\"https:\/\/myendoconsult.com\/learn\/cushings-syndrome\/\">Cushing syndrome<\/a><\/strong> and <strong>intact anterior and posterior pituitary function<\/strong>.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Operative Approach<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Endonasal Route<\/strong> (often with endoscopy):\n<ul class=\"wp-block-list\">\n<li>Via the sphenoid sinus (transsphenoidal approach) and through the floor of the sella.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Histologic Findings<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Corticotroph adenomas are <strong>basophilic<\/strong> and stain positively for ACTH on immunohistochemistry.<\/li>\n\n\n\n<li>Adjacent tissue often shows <strong>Crooke hyaline change<\/strong>, reflecting atrophy of normal corticotrophs.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Cure Rates and Challenges<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Success Rates<\/strong>:\n<ul class=\"wp-block-list\">\n<li><strong>80% to 90%<\/strong> in cases where a microadenoma can be localized preoperatively by MRI or <a href=\"https:\/\/myendoconsult.com\/learn\/inferior-petrosal-sinus-sampling-ipss-testing-procedure-for-evaluating-cushings-syndrome\/\" data-wpil-monitor-id=\"69\">inferior petrosal sinus sampling<\/a>.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Reasons for Lack of Cure<\/strong>:\n<ol class=\"wp-block-list\">\n<li>Very small adenoma, not visualized at surgery.<\/li>\n\n\n\n<li>Inaccessible tumor location (e.g., involvement of the cavernous sinus).<\/li>\n<\/ol>\n<\/li>\n\n\n\n<li><strong>Macroadenomas<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Typically have a lower cure rate (~60%) due to cavernous sinus involvement preventing complete resection.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">PREOPERATIVE AND INTRAOPERATIVE CONSIDERATIONS<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Imaging<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>High-Strength MRI<\/strong> (e.g., 3 tesla) with gadolinium enhancement:\n<ul class=\"wp-block-list\">\n<li>Only about <strong>50%<\/strong> of ACTH-secreting pituitary tumors are large enough to be detected by MRI.<\/li>\n\n\n\n<li>~10% of healthy individuals have an incidental small sellar lesion on MRI, which complicates specificity for a corticotroph adenoma in a patient with <a href=\"https:\/\/myendoconsult.com\/learn\/cushing-syndrome-mnemonic\/\" data-wpil-monitor-id=\"70\">Cushing syndrome<\/a>.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">On the Day of Surgery<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Glucocorticoid Administration<\/strong>:\n<ul class=\"wp-block-list\">\n<li>An <strong>intravenous dose of glucocorticoid<\/strong> (e.g., hydrocortisone, 100 mg) should be given.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Monitoring Serum Cortisol<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Measure the morning serum cortisol concentration <strong>the day after surgery<\/strong> (before any additional exogenous glucocorticoid) to check for short-term cure (low serum cortisol, e.g., &lt;1.8 \u03bcg\/dL).<\/li>\n\n\n\n<li>If acute glucocorticoid withdrawal symptoms occur before results are known, administer \u201cstress dose\u201d glucocorticoids (e.g., hydrocortisone 100 mg IV twice daily).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">POSTOPERATIVE MANAGEMENT<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Glucocorticoid Replacement and Taper<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Initial Taper<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Reduce the steroid dose daily; most patients are discharged on <strong>about twice the standard glucocorticoid replacement<\/strong> (e.g., prednisone 10 mg in the morning and 5 mg at 4 pm daily).<\/li>\n\n\n\n<li>Adjust dose based on severity of preoperative hypercortisolism to avoid severe steroid withdrawal.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Further Reduction<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Slowly taper to a <strong>standard replacement dosage over 4 to 6 weeks<\/strong> after the operation.<\/li>\n\n\n\n<li>Patients typically transition to a single morning dose of a short-acting glucocorticoid (e.g., 15-20 mg hydrocortisone) starting <strong>8 to 12 weeks<\/strong> post-cure.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Assessing Recovery of HPA Axis<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Measure <strong>8 am serum cortisol<\/strong> every 6 weeks (before the morning dose of hydrocortisone).<\/li>\n\n\n\n<li>Cortisol levels eventually rise from undetectable to &gt;10 \u03bcg\/dL. At that point, <strong>taper off the hydrocortisone<\/strong> over 2 weeks.<\/li>\n\n\n\n<li>Most patients require exogenous glucocorticoids for <strong>approximately 12 months<\/strong> after curative surgery.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Resolution of Cushingoid Features<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Signs and symptoms related to Cushing syndrome improve <strong>very slowly<\/strong> over the first 6 months after surgery.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">RECURRENCE AND LONG-TERM FOLLOW-UP<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Risk of Recurrence<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Even with Low Postoperative Cortisol<\/strong>:\n<ul class=\"wp-block-list\">\n<li>If any viable adenomatous corticotroph cells remain, they can multiply over time.<\/li>\n\n\n\n<li><strong>Average time to evident recurrence<\/strong>: 3 to 4 years.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Annual Assessment<\/strong>:\n<ul class=\"wp-block-list\">\n<li>All patients should have <strong>yearly follow-up<\/strong> to detect any return of hypercortisolism.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Thromboembolic Precautions<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Increased Thromboembolic Risk<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Patients with Cushing syndrome have a higher perioperative risk.<\/li>\n\n\n\n<li>Early ambulation and prophylactic measures are encouraged.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">ALTERNATIVE OR ADDITIONAL THERAPIES<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">When Surgery Fails or Is Incomplete<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Repeat Transsphenoidal Surgery<\/strong><\/li>\n\n\n\n<li><strong>Bilateral Laparoscopic Adrenalectomy<\/strong><\/li>\n\n\n\n<li><strong>Radiation Therapy<\/strong> to the sellar region (less frequently used)<\/li>\n\n\n\n<li><strong>Pharmacotherapy<\/strong> to reduce adrenal cortisol production (also less common)<\/li>\n<\/ol>\n\n\n\n\n","protected":false},"excerpt":{"rendered":"<p>CORTICOTROPIN (ACTH)-SECRETING PITUITARY TUMOR Overview and Definition SURGICAL TREATMENT Treatment of Choice Operative Approach Cure Rates and Challenges PREOPERATIVE AND INTRAOPERATIVE CONSIDERATIONS Imaging On&hellip;<\/p>\n","protected":false},"featured_media":0,"template":"","oen_topic_chapter":[682],"class_list":["post-4422274","oen_topic","type-oen_topic","status-publish","hentry","oen_topic_chapter-pituitary-gland"],"_links":{"self":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422274","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic"}],"about":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/types\/oen_topic"}],"version-history":[{"count":5,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422274\/revisions"}],"predecessor-version":[{"id":4422282,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422274\/revisions\/4422282"}],"wp:attachment":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media?parent=4422274"}],"wp:term":[{"taxonomy":"oen_topic_chapter","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic_chapter?post=4422274"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}