This form of hyperaldosteronism is characterized by hypertension secondary to massive adrenal mineralocorticoid production. Like patients with glucocorticoid-remediable aldosteronism (GRA, or FH I; 103900), patients with FH III present with childhood hypertension, elevated aldosteronism levels, and high levels of the hybrid steroids 18-oxocortisol and 18-hydroxycortisol. However, hypertension and aldosteronism in FH III are not reversed by administration of exogenous glucocorticoids and patients require adrenalectomy to control hypertension (Geller et al., 2008).
The cortex is the part of the adrenal gland that makes cortisol Cortisol - a glucocorticoid that controls glucose production and suppresses the immune system , a hormone that is crucial for life. If the patient does not have enough adrenal cortex left, he or she may develop adrenal insufficiency and may need to take steroid medication. ( See What are the potential complications? ) In cases of bilateral adrenal tumors (. a tumor in both adrenal glands) or tumors that are small and clearly not cancer (like aldosterone Aldosterone - a mineralocorticoid that controls blood pressure producing adenomas), some surgeons may perform a cortical-sparing adrenalectomy Cortical-sparing adrenalectomy - an operation to remove the part of an adrenal gland that has the tumor. Also called a partial adrenalectomy. to keep as much of this normal cortex as possible. This usually involves looking at the entire adrenal gland and cutting out just the tumor with a thin rim of normal tissue.