Beyond the three P's: adrenal involvement in MEN1

Area of Science:

• Endocrinology
• Oncology
• Medical Imaging

Background:

• Adrenal lesions (ALs) are frequently observed in patients with multiple endocrine neoplasia type 1 (MEN1).
• Clinical management guidelines for ALs in MEN1 are not well-established.
• Understanding the prevalence and behavior of ALs in MEN1 is crucial for patient care.

Purpose of the Study:

• To investigate the prevalence and clinical outcomes of adrenal lesions in patients with multiple endocrine neoplasia type 1.
• To characterize the imaging features and growth patterns of ALs in MEN1.
• To provide recommendations for the management and work-up of ALs in this population.

Main Methods:

• Retrospective chart review of patients diagnosed with MEN1 between 1990 and 2021.
• Classification of ALs based on imaging findings (unilateral/bilateral, nodular/diffuse, size).
• Analysis of lesion growth, hormonal function, and clinical outcomes.

Main Results:

• Out of 382 MEN1 patients, 89 (23.3%) had ALs, detected at a mean age of 47 years.
• 101 measurable nodular lesions were identified, with a mean size of 17.5 mm; 26.7% were <1 cm.
• Radiographic or clinical progression of ALs was uncommon; malignancy was suspected in cases with significant growth or size. Functional lesions were identified in 15.8% of those tested.

Conclusions:

• Adrenal lesions in MEN1 patients are common but typically show slow progression.
• Malignancy in ALs should be suspected based on lesion size and growth rate.
• Baseline hormonal work-up is recommended; further biochemical testing is unnecessary for non-functioning lesions.