CUSHING'S SYNDROME DUE TO PRIMARY BILATERAL MACRONODULAR ADRENAL HYPERPLASIA AND METACHRONOUS PHEOCHROMOCYTOMA

Area of Science:

• Endocrinology
• Oncology
• Surgical Pathology

Background:

• Primary bilateral macronodular adrenal hyperplasia (PBMAH) is an uncommon cause of ACTH-independent Cushing's syndrome (CS).
• Pheochromocytoma (PCC) is a neuroendocrine tumor arising from the adrenal medulla.
• The simultaneous occurrence of PBMAH and PCC within the same adrenal gland is exceptionally rare.

Observation:

• A 43-year-old woman presented with symptoms of overt ACTH-independent CS.
• She underwent right adrenalectomy for PBMAH, with initial improvement.
• Two years later, she developed recurrent CS and hypertension, leading to left adrenalectomy, revealing a PCC.

Findings:

• The patient experienced metachronous development of PCC after treatment for PBMAH.
• Histopathology confirmed both macronodular adrenal hyperplasia and pheochromocytoma.
• This case represents the first reported instance of overt ACTH-independent CS due to PBMAH coexisting with metachronous PCC.

Implications:

• This case underscores the importance of comprehensive diagnostic re-evaluation for patients with recurrent or persistent ACTH-independent CS.
• It highlights the potential for metachronous development of adrenal tumors, necessitating vigilant long-term follow-up.
• Understanding the coexistence of PBMAH and PCC can inform diagnostic and therapeutic strategies for complex adrenal disorders.