What is the treatment for a functioning adrenaloma?

Treatment of Functioning Adrenaloma

Complete surgical resection (R0 resection) via adrenalectomy is the definitive treatment for functioning adrenalomas, as it provides the only means to achieve long-term cure and resolution of hormone excess. 1

Preoperative Hormonal Management

Before proceeding to surgery, meticulous perioperative management of hormonal abnormalities is critical to prevent complications 1:

• For cortisol-producing adenomas: Patients require postoperative corticosteroid supplementation (hydrocortisone or prednisone) until recovery of the hypothalamus-pituitary-adrenal axis, which may take months 1

• For pheochromocytomas: Alpha-adrenergic blockade must be initiated preoperatively to prevent hypertensive crisis during surgery 2, 3

• For aldosteronomas: Medical management with spironolactone (400-1200 mg/day) can be used preoperatively to control hypertension and hypokalemia 1, 4

Surgical Approach Selection

The choice of surgical technique depends on tumor characteristics 1:

• Laparoscopic adrenalectomy is the preferred approach for:

  • Small tumors (<8 cm) without evidence of invasiveness 1
  • Pheochromocytomas of any size when performed in experienced centers 1
  • All aldosteronomas and cortisol-producing adenomas meeting size criteria 5

• Open adrenalectomy is required for:

  • Tumors >8 cm or with imaging features suspicious for malignancy 1
  • Locally advanced tumors requiring resection of adjacent organs (vena cava, liver, spleen, colon, pancreas, stomach) 1
  • Cases where complete R0 resection is necessary for suspected adrenocortical carcinoma 1

Center Selection

Adrenal surgery should only be performed in specialized centers performing >10 adrenalectomies for adrenal cancer per year 1

Expected Outcomes

Surgical cure rates for functioning tumors are excellent 5:

• Pheochromocytomas: 100% resolution of clinical and biochemical hyperfunction when completely resected 5
• Aldosteronomas: 96% biochemical cure, though 72% may require continued antihypertensive medication for improved blood pressure control 5
• Cortisol-producing adenomas: 100% resolution of hormonal excess 5

Postoperative Follow-up

After complete resection, regular surveillance is mandatory 1:

• Biochemical testing at 14 days post-surgery to confirm complete resection 2
• For pheochromocytomas: Plasma or urinary metanephrines every 3-4 months for 2-3 years, then every 6 months, with lifelong surveillance recommended 1, 2
• For adrenocortical carcinoma: Abdominal CT/MRI and chest CT every 3 months for 2 years, then gradually increased intervals for at least 10 years 1
• Imaging studies should be performed if new symptoms develop or biochemical markers become elevated 1, 2

Special Considerations

High-risk features requiring more aggressive follow-up include 1, 2:

• Tumor size >5 cm
• Extra-adrenal location (paragangliomas)
• SDHB germline mutations
• Elevated plasma methoxytyramine (>3x upper limit)

Common pitfall: Failure to provide adequate corticosteroid replacement after resection of cortisol-producing adenomas can lead to life-threatening adrenal crisis 1