Beyond the three P’s: adrenal involvement in MEN1

作者：U. Clemente-Gutiérrez, Carolina R C Pieterman, Michael S. Lui, Thomas Szabo Yamashita, Andrés Tame-Elorduy, Bernice L. Huang, Aditya S. Shirali, Derek J. Erstad, Jeffrey E. Lee, Sarah B. Fisher, Paul H. Graham, Elizabeth Gardner Grubbs, Steven G. Waguespack, Chaan S. Ng, Nancy D. Perrier · 发表于：Endocrine Related Cancer · 年份：2023 · DOI：10.1530/erc-23-0162 · 被引用次数：7 · 研究领域：Neuroendocrine Tumor Research Advances、Adrenal and Paraganglionic Tumors、Neuroblastoma Research and Treatments

Adrenal lesions (ALs) are often detected in patients with multiple endocrine neoplasia type 1 (MEN1). However, they are not well described in MEN1, making their clinical management unclear. This study examined the prevalence and outcomes of ALs found in MEN1. We performed a retrospective chart review of patients diagnosed with MEN1 from 1990 to 2021. ALs were diagnosed using abdominal or thoracic imaging and classified as being unilateral or bilateral, having single or multiple nodules, and as having diffuse enlargement or not. Measurable nodular lesions were analyzed for their size and growth over time. Patients' clinical and radiographic characteristics were collected. We identified 382 patients with MEN1, 89 (23.3%) of whom had ALs. The mean age at detection was 47 ± 11.9 years. We documented 101 measurable nodular lesions (mean size, 17.5 mm; range, 3-123 mm). Twenty-seven nodules (26.7%) were smaller than 1 cm. Watchful waiting was indicated in 79 (78.2%) patients, of whom 28 (35.4%) had growing lesions. Functional lesions were diagnosed in 6 (15.8%) of 38 that had functional work-up (diagnoses: pheochromocytoma (n = 2), adrenocorticotropic hormone-dependent hypercortisolism (n = 2), hyperandrogenism (n = 1), hyperaldosteronism (n = 1)); surgery was indicated for 5 (83.3%; n = 12 nodules), 2 of whom had bilateral, diffuse adrenal enlargement. Two patients were diagnosed with adrenocortical carcinoma and two with neoplasms of uncertain malignant potential. Radiographic or...