Diagnosis and Medical Management of Adrenal Tumors
Initial Diagnostic Workup
Every patient with a suspected adrenal tumor must undergo both unenhanced CT imaging and comprehensive hormonal evaluation before any surgical intervention, regardless of how benign the tumor appears on imaging. 1, 2
Mandatory Imaging Protocol
- Unenhanced CT of the abdomen is the required first-line imaging study for all adrenal masses, providing critical diagnostic information through Hounsfield unit (HU) measurement 1, 3
- Lesions with **HU <10 strongly indicate benign adenoma**, while HU >10 require further evaluation with second-line imaging or functional studies 1, 4
- Concurrent chest CT should be obtained to evaluate for metastatic disease 1
- Tumors >4 cm carry significantly higher malignancy risk and warrant aggressive workup 5, 3
Mandatory Hormonal Screening (All Patients)
Complete the following hormonal evaluation before any surgical planning:
Cortisol Excess Screening
- 1 mg overnight dexamethasone suppression test is the preferred screening method 1, 2
- Measure plasma ACTH levels 1, 2
- Consider 24-hour urinary free cortisol and basal serum cortisol 1
Catecholamine Excess Screening (Critical for Safety)
- Plasma free metanephrines are the preferred test 1, 2
- Alternative: 24-hour urinary fractionated metanephrines 1, 2
- This test is absolutely mandatory for all patients with adrenal masses >10 HU or any symptoms of catecholamine excess 2
Aldosterone Excess Screening
- Aldosterone-to-renin ratio if hypertension and/or hypokalemia are present 1, 2
- Measure serum potassium levels 2
Androgen/Sex Hormone Screening
- Measure DHEA-S, 17-OH-progesterone, androstenedione, and 17-beta-estradiol in specific cases: young patients, virilization symptoms, or imaging features suggesting adrenocortical carcinoma 2, 3
Management Algorithm Based on Initial Findings
If Lesion Appears Benign on Imaging (HU <10)
- Repeat unenhanced CT in one year 5
- Annual hormone testing for four years 5
- If significant growth (>1 cm increase in diameter) occurs, proceed to surgery 5, 6
If Lesion is Indeterminate (HU >10) or Large (>4 cm)
- Proceed to second-line imaging or functional imaging 5, 1
- FDG-PET is indicated for radiologically indeterminate lesions after second-line imaging 1
- Consider multidisciplinary team decision for further management 5
If Lesion is Obviously Malignant
Features suggesting malignancy include: large size (>4 cm), heterogeneity, evidence of invasion or necrosis 5, 4
- Schedule for open surgical approach if patient is healthy enough for surgery 5
- Strongly recommend open approach (not minimally invasive) for masses >5 cm consistent with adrenocortical carcinoma, as National Cancer Institute data demonstrate significantly higher recurrence rates with minimally invasive approaches 5
If Hormone Hypersecretion is Detected
Pheochromocytoma Management
- Alpha-blocker therapy is mandatory for 1-3 weeks (10-14 days minimum) prior to surgery to prevent intraoperative catecholamine surge 5, 1
- Beta-blockers should NEVER be used before adequate alpha-blockade is established 7
- If necessary, add beta-blocker only after alpha-blockade to control reflex tachycardia 5
- Extend imaging to chest and neck if initial abdominal imaging is negative but biochemistry is positive 1
- Consider genetic testing (approximately 35% are hereditary) and plan lifelong surveillance 1
- For SDHB mutations specifically, use FDG-PET rather than MIBG scintigraphy as it is superior for detecting malignant disease 1
Special Warning: For pure dopamine-secreting tumors, alpha-blockade is NOT indicated and may cause hypotension and cardiovascular collapse 7
Other Hormone-Secreting Tumors
- Immediate surgical removal is indicated for symptomatic hormone-secreting tumors 5
- For adrenocortical carcinoma with endocrine activity, ketoconazole can be used if symptoms are not controlled by tumor mass reduction 8
Critical Safety Pitfalls to Avoid
Never Perform Biopsy Before Excluding Pheochromocytoma
Fine needle biopsy of an adrenal mass must be avoided until pheochromocytoma has been biochemically excluded, as the procedure can trigger a life-threatening hypertensive crisis. 1, 2
- All five major guidelines stipulate that biopsy is of limited clinical value and should not be part of initial workup 5
- Biopsy is only useful in rare instances: hormonally inactive mass with nonbenign imaging where pathology would directly change management, or for staging metastatic disease in patients with known extraadrenal malignancy 5, 3
Do Not Skip Hormonal Evaluation
- Radiological appearance cannot reliably predict hormone secretion status 2, 3
- Even apparently "benign-appearing" tumors require hormonal screening, as subtle hormone production impacts surgical management and perioperative care 1, 2
- Mild autonomous cortisol secretion can be diagnosed in 30-50% of patients with adrenal incidentalomas and is associated with increased cardiovascular morbidity 9
Do Not Use Minimally Invasive Surgery for Large Suspected Carcinomas
- Open surgical approach is strongly recommended for masses >5 cm consistent with adrenocortical carcinoma due to significantly higher recurrence rates with minimally invasive techniques 5
Special Considerations for Adrenocortical Carcinoma
- Approximately 50% of adrenocortical carcinomas secrete cortisol, which aids in diagnosis 5
- Pathological diagnosis requires an experienced pathologist using the Weiss score 1
- Adjunctive treatment with oral mitotane leads to well-documented improvement of survival 8
- Repeated surgery is the mainstay of treatment, either with curative or palliative intention 8
- For unresectable tumors without sufficient response to mitotane, chemotherapy with cisplatin and etoposide (single or in combination with doxorubicin) appears effective 8