PMID- 38108666 OWN - NLM STAT- MEDLINE DCOM- 20240117 LR - 20240210 IS - 1479-6821 (Electronic) IS - 1351-0088 (Print) IS - 1351-0088 (Linking) VI - 31 IP - 2 DP - 2024 Feb 1 TI - Beyond the three P's: adrenal involvement in MEN1. LID - 10.1530/ERC-23-0162 [doi] LID - e230162 AB - 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 clinical progression of ALs is uncommon. Malignancy should be suspected on the basis of a lesion's growth rate and size. A baseline hormonal work-up is recommended, and no further biochemical work-up is suggested when the initial assessment shows nonfunctioning lesions. FAU - Clemente-Gutierrez, Uriel AU - Clemente-Gutierrez U AUID- ORCID: 0000-0002-1591-7845 AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Pieterman, Carolina R C AU - Pieterman CRC AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Lui, Michael S AU - Lui MS AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Yamashita, Thomas Szabo AU - Yamashita TS AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Tame-Elorduy, Andres AU - Tame-Elorduy A AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Huang, Bernice L AU - Huang BL AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Shirali, Aditya S AU - Shirali AS AUID- ORCID: 0000-0002-8132-3391 AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Erstad, Derek J AU - Erstad DJ AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Lee, Jeffrey E AU - Lee JE AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Fisher, Sarah B AU - Fisher SB AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Graham, Paul H AU - Graham PH AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Grubbs, Elizabeth G AU - Grubbs EG AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Waguespack, Steven G AU - Waguespack SG AD - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Ng, Chaan S AU - Ng CS AD - Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. FAU - Perrier, Nancy AU - Perrier N AUID- ORCID: 0000-0001-5933-1005 AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. LA - eng GR - P30 CA016672/CA/NCI NIH HHS/United States PT - Journal Article DEP - 20240116 PL - England TA - Endocr Relat Cancer JT - Endocrine-related cancer JID - 9436481 SB - IM MH - Humans MH - Adult MH - Middle Aged MH - *Multiple Endocrine Neoplasia Type 1/pathology MH - Retrospective Studies MH - *Adrenal Gland Neoplasms/epidemiology MH - *Adrenocortical Carcinoma MH - *Adrenal Cortex Neoplasms PMC - PMC10854230 MID - NIHMS1954900 OTO - NOTNLM OT - adrenal incidentaloma OT - adrenal nodules OT - adrenocortical carcinoma OT - multiple endocrine neoplasia type 1 COIS- Declaration of interest The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported. EDAT- 2023/12/18 12:42 MHDA- 2024/01/17 06:42 PMCR- 2024/02/09 CRDT- 2023/12/18 10:23 PHST- 2023/06/08 00:00 [received] PHST- 2023/12/18 00:00 [accepted] PHST- 2024/01/17 06:42 [medline] PHST- 2023/12/18 12:42 [pubmed] PHST- 2023/12/18 10:23 [entrez] PHST- 2024/02/09 00:00 [pmc-release] AID - ERC-23-0162 [pii] AID - 10.1530/ERC-23-0162 [doi] PST - epublish SO - Endocr Relat Cancer. 2024 Jan 16;31(2):e230162. doi: 10.1530/ERC-23-0162. Print 2024 Feb 1.