Treatment of Cushing's Syndrome in Bilateral Adrenal Disorders
When Cushing's syndrome arises from a bilateral adrenal disorder — such as bilateral macronodular adrenal hyperplasia (BMAH) or primary pigmented nodular adrenal disease (PPNAD) — the underlying pathology shapes the treatment strategy. Both conditions are recognised distinct causes of ACTH-independent hypercortisolism requiring tailored management.
This protocol applies to Cushing's syndrome in the setting of bilateral adrenal disorders, specifically bilateral macronodular adrenal hyperplasia (BMAH) and primary pigmented nodular adrenal disease (PPNAD). The presence of bilateral adrenal involvement and the specific disorder subtype are central to selecting the appropriate treatment path.
Surgical resection of the bilateral adrenal disorder is the recommended treatment. For bilateral macronodular adrenal hyperplasia in which aberrant hormone receptors can be demonstrated, a non-surgical medical alternative may be considered in select cases.
The complete protocol — including surgical approach, patient selection, and the criteria and conditions for medical alternatives — is available in the full regimen below.
References
DOI: 10.1210/jc.2015-1818
- We recommend surgical resection of bilateral adrenal disorders and suggest medical therapy to block aberrant hormone receptors for bilateral macronodular adrenal hyperplasia (BMAH).
- Laparoscopic bilateral adrenalectomy is the definitive treatment of choice and is curative in most cases of primary pigmented nodular adrenal disease, regardless of age.
- Bilateral adrenalectomy, generally via the laparoscopic route, is the surgical treatment of choice, although some advocate for selective removal of the larger adrenal in older patients in whom nonfunctional tumors are a possibility, especially if there is only a single nodule on each side.
- BMAH can be treated by an appropriate antagonist if aberrant receptors are demonstrated that clearly couple to cortisol release, but the detailed workup of such patients is a research undertaking beyond the scope of these guidelines.