PMID- 37668926 OWN - NLM STAT- MEDLINE DCOM- 20231002 LR - 20240201 IS - 1559-0100 (Electronic) IS - 1355-008X (Linking) VI - 82 IP - 2 DP - 2023 Nov TI - Thymoma in multiple endocrine neoplasia type 1: a case report and systematic review. PG - 442-449 LID - 10.1007/s12020-023-03440-5 [doi] AB - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome that combines endocrine and non-endocrine tumors. Thymic neuroendocrine tumors are uncommon components that predict poor prognosis in patients with MEN1. We aimed to summarize the clinical characteristics of thymoma in MEN1 by reviewing the current reports from the literature. METHODS: A patient with multiple endocrine neoplasia type 1 (parathyroid hyperplasia, pituitary adenoma, and insulinoma) was found to have a 2 x 1.5 cm thymic mass during long-term follow-up. Thoracoscope surgery was performed, and a histopathology examination revealed WHO Type B3 thymoma. A pathogenic mutation of c.783 + 1G > A in the MEN1 gene was identified. We further searched PubMed and EMBASE for thymoma in association with MEN1. RESULTS: A comprehensive overview of the literature concerning characteristics of MEN1-related thymoma was summarized. Clinical characteristics and differences between thymoma and thymic carcinoid are highlighted. CONCLUSIONS: Besides carcinoid, other tumors, including thymoma, need to be identified for thymic space-occupying lesions in MEN1 patients. The impact of thymoma on the long-term prognosis of MEN1 patients needs further investigation. CI - (c) 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. FAU - Gao, Yuting AU - Gao Y AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. FAU - Li, Ran AU - Li R AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. FAU - Wu, Lingge AU - Wu L AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. FAU - Yang, Hongbo AU - Yang H AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. FAU - Mao, Jiangfeng AU - Mao J AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. FAU - Zhao, Weigang AU - Zhao W AUID- ORCID: 0000-0002-1472-7118 AD - Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, 100730, Beijing, China. xiehezhaoweigang@163.com. LA - eng PT - Case Reports PT - Journal Article PT - Systematic Review DEP - 20230905 PL - United States TA - Endocrine JT - Endocrine JID - 9434444 SB - IM MH - Humans MH - *Multiple Endocrine Neoplasia Type 1/complications/genetics/diagnosis MH - *Thymoma/diagnostic imaging/complications MH - *Thymus Neoplasms MH - *Carcinoid Tumor/pathology MH - *Pancreatic Neoplasms/diagnosis OTO - NOTNLM OT - MEN1 OT - Mediastinal tumors OT - Multiple endocrine neoplasia type 1 OT - Thymic neuroendocrine tumors OT - Thymoma EDAT- 2023/09/05 12:41 MHDA- 2023/10/02 06:42 CRDT- 2023/09/05 11:20 PHST- 2023/05/02 00:00 [received] PHST- 2023/06/21 00:00 [accepted] PHST- 2023/10/02 06:42 [medline] PHST- 2023/09/05 12:41 [pubmed] PHST- 2023/09/05 11:20 [entrez] AID - 10.1007/s12020-023-03440-5 [pii] AID - 10.1007/s12020-023-03440-5 [doi] PST - ppublish SO - Endocrine. 2023 Nov;82(2):442-449. doi: 10.1007/s12020-023-03440-5. Epub 2023 Sep 5.