Primary bilateral macronodular adrenal hyperplasia: A rare case report of Cushing syndrome and review of literature.

Rationale: Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing syndrome (CS), accounting for <2% of CS cases. Diagnosing PBMAH can be difficult and challenging for clinicians.

Patient Concerns: We report a 52-year-old female, a patient with a history of intermittent fever for 3 years. She presented with nausea, headache, and dizziness for several days, along with fatigue, myalgia, muscle weakness, exertional dyspnea, hoarseness, spontaneous bruising over the past several months, and long-term psychological complaints. Additionally, we observed periorbital and facial edema, right lower quadrant tenderness, and abdominal striae during the examination.

Diagnoses: Her laboratory results showed increased cortisol and suppressed ACTH, and an abdominal CT scan revealed 2 heterogeneous masses in the adrenal glands. These findings led us to the diagnosis of PBMAH in this patient. The existence of aberrant receptors was evaluated, and the tests were negative.

Interventions: The patient underwent left adrenalectomy and corticosteroid therapy after the surgery. Her clinical complaints improved after the surgery.

Outcomes: However, her dependency on corticosteroids was not transient after unilateral adrenalectomy, and she still needs glucocorticoid supplementation 1 year after surgery.

Lessons: This patient is a case of PBMAH who presented with fever and CS symptoms and underwent unilateral adrenalectomy. Interestingly, she had suppressed cortisol levels for at least 1 year after the unilateral adrenalectomy. Therefore, we suggest further research on the most effective treatment strategies for PBMAH.