The Role of Cortisol Secretion in Pheochromocytomas and Paragangliomas: Clinical and Perioperative Implications.

Background: Pheochromocytomas and paragangliomas (PPGLs) are tumors marked by excessive catecholamine secretion. Patients with pheochromocytomas may have elevated plasma glucocorticosteroid concentrations. This study aimed to evaluate the prevalence, clinical implications, and perioperative outcomes of autonomous cortisol secretion in patients with PPGLs.

Design: This was a retrospective cohort study conducted across two tertiary endocrinology centers, including patients with PPGLs who underwent adrenalectomy or extra-adrenal surgery for paragangliomas.

Methods: Patients were divided based on the 1 mg dexamethasone suppression test (DST) results into suppressive and non-suppressive groups (above or below 1.8 µg/dL [50 nmol/L]). Data on clinical characteristics, biochemical markers, tumor features, perioperative outcomes, and follow-up were analyzed.

Results: Among 106 patients, 24.5% exhibited non-suppressive cortisol concentrations post-DST. These patients were older (median age: 66 vs. 56 years, P<0.001), predominantly female (84.6% vs. 48.8%, P=0.001), and presented with larger tumors (5.2 vs. 4.0 cm, P<0.05). Diabetes was more common in the non-suppressive group both before adrenalectomy/surgery (50.0% vs. 26.8%, P<0.05) and after (33.3% vs. 12.7%, P<0.05). The non-suppressive group had higher urinary and plasma metanephrine concentrations, lower DHEAS concentrations, and more cardiovascular diseases. Perioperative complications, including blood loss, conversion to open surgery, and prolonged hospital stays, were more frequent in the non-suppressive group (P<0.05).

Conclusions: One-quarter of patients with PPGLs exhibit autonomous cortisol secretion, associated with larger tumors, higher diabetes prevalence, and increased perioperative risks. Routine DST screening may improve preoperative management and offer insights into the impact of cortisol on PPGLs outcomes.