Adrenal medullary hyperplasia (AMH) presents with milder signs and symptoms compared with pheochromocytomas, with the exception of Cushing symptoms, according to study results published in the Journal of the Endocrine Society.
Data for AMH are scarce, given the rarity of the condition, which can make it difficult to diagnose. The researchers investigated a large cohort of AMH cases to characterize their presentation, management, and long-term outcomes compared with pheochromocytoma. The retrospective study included patients with AMH (n=19) and patients who had pheochromocytoma without AMH (n=95), who served as a control group. The researchers analyzed AMH cases without pheochromocytoma (n=7) and those with pheochromocytoma (n=12) separately.
Of the AMH cases, 11% (n=2) had no concurrent pheochromocytoma or adrenocortical adenoma. The researchers found that 58% of patients with AMH had a genetic syndrome (mainly multiple endocrine neoplasia type 2A) compared with only 4% of those with pheochromocytomas without AMH (P <.001).
Patients with AMH without pheochromocytoma had milder symptoms compared with the control group, including lower rates of headaches, palpitations, sweating, feeling “warm flush,” nausea, weight loss, tremor, paroxysmal symptoms, and orthostatic symptoms. However, those with AMH only were significantly more likely to have Cushing symptoms compared with cases of pheochromocytoma.
Out of the AMH cases, 37% presented as an incidentaloma compared with 67% of pheochromocytomas without AMH.
Surgical management of AMH compared with pheochromocytoma was similar. However, 26% of patients with AMH did not receive alpha-blockers before surgery because AMH was not suspected. The researchers suggest that if AMH is suspected, preoperative and postsurgical management should be the same as with pheochromocytoma. After adrenalectomy, patients with AMH without pheochromocytoma showed less improvement in blood pressure compared with those with AMH and pheochromocytoma or pheochromocytoma only.
During 11.2±9.4 years of follow-up, 32% of participants with AMH developed a new pheochromocytoma.
“Short- and long-term outcomes seem favorable, but AMH needs life-long follow-up similar to [pheochromocytoma],” the researchers wrote.
Falhammer H, Stenman A, Calissendorff J, Juhlin CC. Presentation, treatment, histology and outcomes in adrenal medullary hyperplasia compared to pheochromocytoma [published online June 11, 2019]. J Endocrine Soc. doi:10.1210/js.2019-00200