PMID- 38161202 OWN - NLM STAT- Publisher LR - 20231231 IS - 1720-8386 (Electronic) IS - 0391-4097 (Linking) DP - 2023 Dec 31 TI - The risk of concurrent malignancies in patients with multiple endocrine neoplasia type 1: insights into clinical characteristics of those with multiple endocrine neoplasia type 1. LID - 10.1007/s40618-023-02288-w [doi] AB - OBJECTIVE: Summarize and analyze the characteristics of patients with Multiple Endocrine Neoplasia type 1 (MEN-1) who were diagnosed with malignant tumors that do not belong to MEN-1 components. METHODS: Clinical data from patients with MEN-1 who visited Peking Union Medical College Hospital between April 2012 and April 2022 were collected. We compared the clinical characteristics of patients with malignant tumors outside of their MEN-1 components to those without additional tumors. MEN-1 gene testing was performed on most of these patients using Sanger sequencing, whole-exome sequencing, or MLPA. RESULTS: A total of 221 MEN-1 patients were diagnosed, of which 23 (10.40%) were found to have malignant tumors that did not belong to MEN-1 components, including papillary thyroid carcinoma (PTC) (4.52%), breast cancer (1.81%), urologic neoplasms (1.35%), primary hepatic carcinoma (PCC) (0.09%), meningeal sarcoma (0.05%), glioblastoma (0.05%), cervical cancer (0.05%), and lung carcinoma (0.05%). MEN-1 gene mutations were identified in 11 patients, including missense mutations, frameshift mutations, and splice mutations. The prevalence of each endocrine neoplasm, particularly gastroenteropancreatic neuroendocrine tumor, was higher in MEN-1 patients with other malignant tumors compared to MEN-1 patients without malignant tumors. CONCLUSION: Our retrospective study revealed a higher incidence of non-MEN-1 component malignant tumors in MEN-1 patients, especially breast cancer, PTC, and urologic neoplasms. These patients also exhibit more severe clinical phenotypes of MEN-1. CI - (c) 2023. The Author(s), under exclusive licence to Italian Society of Endocrinology (SIE). FAU - Zhao, Yu Xing AU - Zhao YX AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Wang, Ou AU - Wang O AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Song, An AU - Song A AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Wang, Lin Jie AU - Wang LJ AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Gong, Feng Ying AU - Gong FY AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Duan, Lian AU - Duan L AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Yang, Hong Bo AU - Yang HB AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Pan, Hui AU - Pan H AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. FAU - Zhu, Hui Juan AU - Zhu HJ AUID- ORCID: 0000-0001-5172-6870 AD - Key Laboratory of, Endocrinology of National Health Commission, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China. shengxin2004@163.com. AD - Chinese Research Center for Behavior Medicine in Growth and Development, Beijing, 100730, Beijing, China. shengxin2004@163.com. LA - eng GR - 2021-I2M-1-023/Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences/ PT - Journal Article DEP - 20231231 PL - Italy TA - J Endocrinol Invest JT - Journal of endocrinological investigation JID - 7806594 SB - IM OTO - NOTNLM OT - Breast cancer OT - MEN-1 OT - Multiple endocrine neoplasia OT - Papillary thyroid carcinoma EDAT- 2024/01/02 11:45 MHDA- 2024/01/02 11:45 CRDT- 2023/12/31 23:03 PHST- 2023/07/11 00:00 [received] PHST- 2023/12/17 00:00 [accepted] PHST- 2024/01/02 11:45 [medline] PHST- 2024/01/02 11:45 [pubmed] PHST- 2023/12/31 23:03 [entrez] AID - 10.1007/s40618-023-02288-w [pii] AID - 10.1007/s40618-023-02288-w [doi] PST - aheadofprint SO - J Endocrinol Invest. 2023 Dec 31. doi: 10.1007/s40618-023-02288-w.