PMID- 37390813 OWN - NLM STAT- MEDLINE DCOM- 20231120 LR - 20231129 IS - 1945-7197 (Electronic) IS - 0021-972X (Linking) VI - 108 IP - 12 DP - 2023 Nov 17 TI - Traits of Patients With Pituitary Tumors in Multiple Endocrine Neoplasia Type 1 and Comparing Different Mutation Status. PG - e1532-e1541 LID - 10.1210/clinem/dgad387 [doi] AB - CONTEXT: Recent studies suggest that the clinical characteristics and biological behavior of pituitary tumors (PITs) in patients with multiple endocrine neoplasia type 1 (MEN1) may not be as aggressive as previously reported. Increased imaging of the pituitary as recommended by screening guidelines identifies more tumors, potentially at an earlier stage. However, it is unknown if these tumors have different clinical characteristics in different MEN1 mutations. OBJECTIVE: To assess characteristics of patients with MEN1 with and without PITs, and compare among different MEN1 mutations. METHODS: Data of patients with MEN1 in a tertiary referral center from 2010 to 2023 were retrospectively analyzed. RESULTS: Forty-two patients with MEN1 were included. Twenty-four patients had PITs, 3 of which were invasive and managed with transsphenoidal surgery. One PIT enlarged during follow-up. Patients with PITs had a higher median age at MEN1 diagnosis than those without PITs. MEN1 mutations were identified in 57.1% of patients, including 5 novel mutations. In patients with PITs, those with MEN1 mutations (mutation+/PIT+ group) had more additional MEN1-associated tumors than those without (mutation-/PIT+ group). The mutation+/PIT+ group had a higher incidence of adrenal tumors and a lower median age at initial manifestation of MEN1 than the mutation-/PIT+ group. The most common neuroendocrine neoplasm was nonfunctional in the mutation+/PIT+ group and insulin-secreting in the mutation-/PIT+ group. CONCLUSION: This is the first study comparing characteristics of patients with MEN1 with and without PITs harboring different mutations. Patients without MEN1 mutations tended to have less organ involvement and it might be reasonable for them to receive less intensive follow-up. CI - (c) The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. FAU - Lin, Kuan-Yu AU - Lin KY AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Yunlin Branch, Douliu City 640203, Taiwan. FAU - Kuo, Yu-Ting AU - Kuo YT AD - Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Cheng, Mei-Fang AU - Cheng MF AD - Department of Nuclear Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. AD - Institute of Environmental and Occupational Health Sciences, National Taiwan University, Taipei 100025, Taiwan. FAU - Chen, Pei-Lung AU - Chen PL AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. AD - Graduate Institute of Medical Genomics and Proteomics, National Taiwan University College of Medicine, Taipei 100233, Taiwan. AD - Department of Medical Genetics, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Wang, Hsiu-Po AU - Wang HP AD - Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Cheng, Tsu-Yao AU - Cheng TY AD - Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. AD - Department of Laboratory Medicine, National Taiwan University Cancer Center, Taipei 106037, Taiwan. FAU - Chang, Chia-Hsuin AU - Chang CH AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Kao, Hsiang-Fong AU - Kao HF AD - Department of Medical Oncology, National Taiwan University Cancer Center, Taipei 106037, Taiwan. FAU - Yang, Shih-Hung AU - Yang SH AD - Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Li, Hung-Yuan AU - Li HY AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Lin, Chia-Hung AU - Lin CH AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Hsin-Chu Branch, Hsin-Chu 302058, Taiwan. FAU - Chou, Yuh-Tsyr AU - Chou YT AD - Graduate Institute of Medical Genomics and Proteomics, National Taiwan University College of Medicine, Taipei 100233, Taiwan. FAU - Chung, An-Ko AU - Chung AK AD - Graduate Institute of Medical Genomics and Proteomics, National Taiwan University College of Medicine, Taipei 100233, Taiwan. FAU - Wu, Wan-Chen AU - Wu WC AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Lu, Jin-Ying AU - Lu JY AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. FAU - Wang, Chih-Yuan AU - Wang CY AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. AD - Department of Internal Medicine, National Taiwan University College of Medicine, Taipei 100233, Taiwan. FAU - Hsih, Wen-Hui AU - Hsih WH AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Yunlin Branch, Douliu City 640203, Taiwan. FAU - Wen, Chen-Yu AU - Wen CY AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Yunlin Branch, Douliu City 640203, Taiwan. FAU - Yang, Wei-Shiung AU - Yang WS AD - Department of Internal Medicine, National Taiwan University College of Medicine, Taipei 100233, Taiwan. AD - Graduate Institute of Clinical Medicine, National Taiwan University College of Medicine, Taipei 100233, Taiwan. FAU - Shih, Shyang-Rong AU - Shih SR AUID- ORCID: 0000-0001-7424-1358 AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei 100225, Taiwan. AD - Department of Internal Medicine, National Taiwan University College of Medicine, Taipei 100233, Taiwan. AD - Center of Anti-Aging and Health Consultation, National Taiwan University Hospital, Taipei 100225, Taiwan. LA - eng GR - Liver Disease Prevention and Treatment Research Foundation, Taiwan/ GR - National Taiwan University Hospital/ GR - National Taiwan University/ GR - Wong-Yuan Endocrine Fund in Taiwan/ PT - Journal Article PL - United States TA - J Clin Endocrinol Metab JT - The Journal of clinical endocrinology and metabolism JID - 0375362 SB - IM MH - Humans MH - *Multiple Endocrine Neoplasia Type 1/pathology MH - *Pituitary Neoplasms/epidemiology/genetics/pathology MH - Retrospective Studies MH - Mutation MH - Pituitary Gland/pathology OTO - NOTNLM OT - MEN1 gene OT - multiple endocrine neoplasia type 1 OT - pituitary tumors EDAT- 2023/07/01 11:41 MHDA- 2023/11/20 06:54 CRDT- 2023/06/30 18:13 PHST- 2023/04/06 00:00 [received] PHST- 2023/11/20 06:54 [medline] PHST- 2023/07/01 11:41 [pubmed] PHST- 2023/06/30 18:13 [entrez] AID - 7216398 [pii] AID - 10.1210/clinem/dgad387 [doi] PST - ppublish SO - J Clin Endocrinol Metab. 2023 Nov 17;108(12):e1532-e1541. doi: 10.1210/clinem/dgad387.