Table 2.
Therapeutic interventions and outcomes of Mayo Clinic and case-reported patients with coexisting pheochromocytoma and primary aldosteronism
| Patient number | Type of adrenalectomy | Final histopathology | Postoperative catecholamine excess improved | Postoperative biochemical outcome of PAa | Postoperative hypertensionb | Postoperative potassium (mEq/L) |
|---|---|---|---|---|---|---|
| 1 | Right total | Right: 3.8 cm composite PHEO with focal areas of ganglioneuroblastoma differentiation and hemorrhage, a discrete cortical nodule in the zona glomerulosa | Yes | PAC: 16 | Yes | Potassium normal |
| PRA: 0.6 | BP normal | On MRA | ||||
| Bilateral PA on AVS | On anti-hypertensives | |||||
| Persistent PA | ||||||
| 2 | Right total | Right: 3.5 cm PHEO | Yes | PAC: 14 | Yes | Potassium 4.2 |
| PRA: 0.6 | BP 170/80 | On MRA | ||||
| Persistent PA | On anti-hypertensives | |||||
| 3 [22] | Right total and left subtotal | Right: 2 PHEOs (4 cm and 0.9 cm); mild cortical hyperplasia with focal occurrence of cells with deeply eosinophilic cytoplasm in the superficial cortex, nuclear enlargement, and cytoplasmic globules, reminiscent of aldactone bodies; 3 mm cavernous hemangioma also present in cortex | Yes | PAC: 18 | No | Potassium normal |
| PRA: 1.2 | BP 122/80 | Not on MRA | ||||
| 24-hour urinary aldosterone: 2.7 mcg | Not on anti-hypertensives | |||||
| Resolved PA | ||||||
| Left: 1.5 cm PHEO; mild cortical hyperplasia with 2 cortical nodules | ||||||
| 4 | Bilateral subtotal | Right: 1.5 cm PHEO; multinodular cortical hyperplasia, with multiple nodules up to 0.8 cm | Yes | PAC: 2 | Yes | Potassium 4.2 |
| PRA: normal | BP 120/70 | Not on MRA | ||||
| Left: Multinodular cortical hyperplasia, with nodules up to 1.0 cm | ||||||
| Resolved PA | On anti-hypertensives | |||||
| 5 | Right total | Right: 2.5 cm PHEO, R macronodular hyperplasia of adrenal cortex | Yes | PAC: 13 | Yes | Potassium 5.1 |
| PRA: 0.2 | BP 180/100 | On MRA | ||||
| Persistent PA | On anti-hypertensives | |||||
| Left total adrenalectomyc was performed years later due to autonomous cortisol secretion, which showed diffuse and nodular adrenocortical hyperplasia | ||||||
| 6 [5] | Left total | Left: 0.7 cm PHEO; 2 cm cortical tumor; 0.2 cm and 0.1 cm cortical nodule composed of lipid laden clear cells. No hyperplastic changes in remaining cortex, only focal lymphocytic and plasma cell infiltration at the zona glomerulosa | Yes | PAC: 10.4 | Unknown | Potassium normal |
| PRA: 3.2 | BP 130/80 | Unknown if on MRA | ||||
| Resolved PA | Unknown if on anti-hypertensives | |||||
| 7 [6] | Right total | Right: Adrenal medulla totally occupied by PHEO; 1.0 cm adrenocortical adenoma | Yes | PAC: 5 | Unknown | Unknown potassium or if on MRA |
| PRA: unknown | BP normal | |||||
| Resolved PA | Unknown if on anti-hypertensives | |||||
| 8 [7] | Right total | Right: 3.3 cm PHEO extending up to the capsule and merging with a nodular hyperplastic area of zona fasciculata; in other areas of cortex, hyperplasia of zona glomerulosa was seen; karyotype abnormalities of long-term tissue culture of cortical cells | Yes | PAC: unknown | Unknown | Unknown potassium or if on MRA |
| PRA: unknown | BP normal | |||||
| Fludrocortisone failed to suppress PAC | Unknown if on anti-hypertensives | |||||
| Bilateral PA on AVS | ||||||
| Persistent PA | ||||||
| 9 [7] | Left total | Left: 5.5 cm PHEO; 1 cm cortical adenoma with many other areas of hyperplastic adrenal cortex including nodules up to 0.3 cm diameter which histologically resembled zona fasciculata | Yes | PAC: unknown | Unknown | Unknown potassium or if on MRA |
| PRA: unknown | Unknown BP or if on anti-hypertensives | |||||
| Normal PAC/PRA | ||||||
| Resolved PA | ||||||
| 10 [8] | Right total | Right: mass involved both cortex and medulla and was consistent with PHEO and adrenal cortical adenoma | Unknown | Blood pressure and hypokalemia resolved | No | Potassium 4.2 |
| Resolved PA | BP 110/60 | Not on MRA | ||||
| Not on anti-hypertensives | ||||||
| 11 [9] | Bilateral subtotal | Right: 5.0 cm PHEO; nonnodular yellow cortical layer contained an adrenocortical adenoma with clear cells in a honeycomb pattern | Yes | PRA: normal | Unknown | Unknown potassium or if on MRA |
| PAC: normal | BP normal | |||||
| Resolved PA | Unknown if on anti-hypertensives | |||||
| Left: 1.9 cm yellowish mass that was consistent with adrenocortical adenoma composed of clear cells | ||||||
| 12 [10] | Right total | Right: 10 cm PHEO | Unknown | Previous AVS suggested left-sided PA | Yes | Potassium normal |
| BP normal | ||||||
| Treated with right adrenalectomy for PHEO | On anti-hypertensives | On MRA | ||||
| Persistent PA | ||||||
| 13 [11] | Left total | Left: 1.9 cm PHEO; 2.4 cm yellow round mass composed of clear cells, P450C17 not expressed (suggesting tumor not secreting cortisol) though all other steroid synthetases (p450SCC, 3BHSD, p450c21, and p450c11) were expressed, suggesting aldosterone production. Zona glomerulosa of normal adrenal gland showed hyperplasia with no expression of 3B-HSD, which was construed as a finding of paradoxical hyperplasia associated with aldosterone overproduction. No significant atrophy in zona fasciculata or reticularis (no HPA axis suppression so likely no long-term cortisol production) | Yes | PAC: 4.28 | No | Potassium 4 |
| PRA: 0.3 | BP 120/70 | Not on MRA | ||||
| Resolved PA | Not on anti-hypertensives | |||||
| 14 [12] | Right total | Left: 2.2 cm PHEO, 1.1 cm yellowish tumor composed of lipid laden clear cells, diagnosed as adrenocortical adenoma (mRNA evidence of aldosterone production | Yes | PAC: 52.7 | Yes | Potassium 3 |
| PRA: 0.1 | BP 120/70 | On MRA | ||||
| Bilateral PA on AVS | On anti-hypertensives | |||||
| Persistent PA | ||||||
| 15 [13] | Right total | Right: 4 cm PHEO | Yes | PAC: unknown | No | Unknown potassium |
| PRA: unknown | BP normal | Not on MRA | ||||
| Normal PAC/PRA | Not on anti-hypertensives | |||||
| Resolved PA |
Abbreviations: MRA, mineralocorticoid receptor antagonist; PA, primary aldosteronism; PAC, plasma aldosterone concentration; PHEO, pheochromocytoma; PRA, plasma renin activity.
aPostoperative biochemical outcomes of PA described persistence or resolution of PA; PAC normal range <10 ng/dL; PRA normal range <1 ng/mL/h.
bHypertension was defined as systolic blood pressure (SBP) ≥140 mm Hg, diastolic blood pressure (DBP) ≥90 mm Hg, and/or requiring antihypertensive therapy.
cPatient 5 required lifelong corticosteroid and mineralocorticoid replacement after undergoing left total adrenalectomy for subclinical glucocorticoid secretory autonomy 3 years after initial right total adrenalectomy.