Primary Bilateral Macronodular Adrenal Hyperplasia: A Rare Cause Of Cushing Syndrome
October 2023
in “
Journal of the Endocrine Society
”
Cushing syndrome primary bilateral macronodular adrenal hyperplasia PBMAH clinical hypercortisolism bilateral adrenal adenomas adrenocortical adenoma unilateral adrenalectomy bilateral adrenalectomy adrenal insufficiency Cushing's disease adrenal gland hyperplasia adrenal gland tumors adrenal gland surgery
TLDR A rare case of Cushing syndrome was successfully treated with one-sided adrenal gland removal, suggesting personalized treatment is important.
The document describes a rare case of primary bilateral macronodular adrenal hyperplasia (PBMAH), which accounts for less than 2% of Cushing syndrome (CS) cases. A 42-year-old female patient with a history of arterial hypertension presented with abdominal pain and signs of clinical hypercortisolism, including significant weight gain, increased abdominal circumference, muscle weakness, and sudden facial and abdominal hair growth. Imaging and laboratory tests confirmed the presence of bilateral adrenal adenomas and clinical hypercortisolism. Despite bilateral adrenalectomy being the standard treatment, the patient underwent unilateral left-sided adrenalectomy due to the lower risk of complications and potential for adrenal insufficiency. The surgery was successful, with the pathology report confirming adrenocortical adenoma. Unilateral adrenalectomy has shown more than 90% initial remission rates with a low recurrence risk of 10-15%, and about one-third of patients experience transient adrenal insufficiency. This case highlights the need for personalized treatment approaches in managing PBMAH to balance therapeutic benefits against the risks of complications.