What is the management approach for a patient with bilateral adrenal tumors and a brain tumor?

Management of Bilateral Adrenal Tumors with Brain Tumor

The management of a patient with bilateral adrenal tumors and a brain tumor requires a comprehensive multidisciplinary approach with surgical resection of all tumors when feasible, as this offers the best chance for improved mortality and quality of life outcomes. 1

Initial Diagnostic Workup

Hormonal Evaluation

• All adrenal masses require complete hormonal evaluation regardless of imaging findings:
  • 1mg overnight dexamethasone suppression test (cortisol ≤50 nmol/L or ≤1.8 µg/dL indicates normal suppression) 2
  • Plasma or 24-hour urinary metanephrines (to rule out pheochromocytoma) 1
  • Aldosterone-to-renin ratio (for primary aldosteronism) 1
  • Consider serum DHEAS levels (elevated in adrenocortical carcinoma) 3

Imaging Studies

• Adrenal imaging:

  • Non-contrast CT (benign adenomas typically have HU ≤10) 3
  • Contrast-enhanced CT with washout protocol (>60% washout at 15 minutes suggests benign lesion) 3
  • Consider FDG-PET/CT for suspected malignancy 1

• Brain tumor evaluation:

  • MRI with contrast is the preferred imaging modality
  • Consider additional functional imaging based on suspected tumor type

Differential Diagnosis of Bilateral Adrenal Tumors

Common etiologies include:

1. Bilateral pheochromocytomas (33% of bilateral adrenal tumors) 4
2. Primary adrenal lymphoma (22%) 4
3. Nonfunctioning cortical adenomas (22%) 4
4. Metastatic disease (11%) 4
5. Primary aldosteronism with bilateral hyperplasia 1
6. Cushing syndrome from bilateral cortisol-producing adenomas 1

Management Algorithm

Step 1: Determine Functionality

• If pheochromocytoma is confirmed:

  • Preoperative alpha-blockade followed by beta-blockade
  • Surgical resection of both tumors when feasible 1
  • For inoperable disease: Consider 131I-MIBG therapy, locoregional procedures, or CVD chemotherapy 1

• If cortisol-producing (Cushing syndrome):

  • Surgical resection if unilateral dominant source
  • For bilateral disease with asymmetric cortisol production: Remove the most active side 1
  • For symmetric production: Medical management with ketoconazole (400-1200 mg/day) or mitotane 1

• If aldosterone-producing:

  • Unilateral adenoma: Laparoscopic adrenalectomy
  • Bilateral hyperplasia: Medical management with spironolactone or eplerenone 1

Step 2: Assess Risk of Malignancy

• High risk features for adrenocortical carcinoma:

  • Size >4-5 cm
  • Irregular margins
  • Heterogeneous appearance
  • HU >10 on non-contrast CT
  • Poor contrast washout

• If malignancy suspected:

  • Complete surgical extirpation (R0 resection) with locoregional lymphadenectomy 1
  • For inoperable ACC: EDP-M chemotherapy (etoposide, doxorubicin, cisplatin plus mitotane) for fit patients with high tumor burden 1
  • Consider adjuvant mitotane for patients with incomplete resection or high-risk features (Ki67>10%) 1

Step 3: Brain Tumor Management

• Coordinate timing of surgeries with neurosurgery
• Generally, address hormone-secreting tumors first, particularly pheochromocytoma
• For metastatic disease to brain from adrenal primary: Consider systemic therapy, stereotactic radiosurgery, or surgical resection based on number and location of lesions

Special Considerations

Bilateral Adrenalectomy

• If bilateral complete adrenalectomy is required, lifelong glucocorticoid and mineralocorticoid replacement therapy will be necessary
• Consider cortical-sparing adrenalectomy when appropriate to preserve adrenal function

Follow-up Recommendations

• For resected adrenal tumors: Clinical, imaging, and biochemical monitoring for at least 10 years 1
• For inoperable disease: Evaluation every 2-4 months for ACC and every 3-6 months for pheochromocytoma during the first year 1
• For malignant pheochromocytoma: Lifelong surveillance is recommended 1

Common Pitfalls to Avoid

1. Failing to rule out pheochromocytoma before any intervention (can cause life-threatening crisis)
2. Performing needle biopsy of potentially resectable adrenal masses (contraindicated and potentially harmful) 1
3. Delaying surgical management of suspected adrenocortical carcinoma
4. Overlooking mild autonomous cortisol secretion, which can impact perioperative management
5. Neglecting to coordinate timing of surgeries between adrenal and brain tumors

This management approach prioritizes identification and treatment of conditions with the highest impact on mortality and quality of life, while providing a structured pathway for evaluation and treatment.