Persistent or recurrent Cushing's disease: Surgical adrenalectomy
- Lynnette K Nieman, MD
Lynnette K Nieman, MD
- Section Editor — Adrenal Disease
- Senior Investigator
- Bethesda, MD
The treatment of choice for patients with Cushing's disease (corticotropin [ACTH]-secreting pituitary tumor) is transsphenoidal surgery and resection of the pituitary tumor. However, for patients with persistent or recurrent Cushing's disease, adrenalectomy, which can be achieved either surgically or medically (with mitotane), is sometimes recommended for definitive cure.
Surgical adrenalectomy for persistent or recurrent Cushing's disease is reviewed here. An overview of the treatment of Cushing's syndrome, medical therapy of hypercortisolism, and primary therapy for Cushing's disease are reviewed separately. (See "Overview of the treatment of Cushing's syndrome" and "Medical therapy of hypercortisolism (Cushing's syndrome)" and "Primary therapy of Cushing's disease: Transsphenoidal surgery and pituitary irradiation".)
GENERAL APPROACH TO CUSHING'S DISEASE
Cushing's disease is caused by pituitary corticotropin (ACTH)-secreting tumors. These corticotroph tumors are almost always benign and are usually microadenomas (ie, <10 mm in diameter). The progressive stages of treatment that may be required to cure a patient of Cushing's disease are shown in the algorithm (algorithm 1). Our approach is largely consistent with the Endocrine Society Clinical Practice Guideline .
Transsphenoidal microadenomectomy is currently the treatment of choice for Cushing's disease. A neurosurgeon with expertise in transsphenoidal surgery for Cushing's patients can achieve an initial cure rate of 80 to 90 percent with microadenomas but less than 60 percent with macroadenomas. A meta-analysis of 18 reports since 1995 showed an overall initial cure rate for micro- and macroadenomas combined of 79 percent . It is important to recognize that patients who are initially "cured" should be considered to be in remission rather than cured as some will recur. Overall, remission from centers with less experience may be much lower, approaching only 50 percent long-term cure . (See "Primary therapy of Cushing's disease: Transsphenoidal surgery and pituitary irradiation", section on 'Transsphenoidal surgery'.)
For patients with clear persistent disease after transsphenoidal surgery, there are five therapeutic options:
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- GENERAL APPROACH TO CUSHING'S DISEASE
- SURGICAL ADRENALECTOMY
- Choice of procedure
- - Surgical approach
- Hormone replacement
- Postoperative management
- - Monitoring
- Quality of life
- Nelson syndrome
- - Incidence
- - Pathogenesis
- - Biochemical criteria
- - Prevention
- - Treatment
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS