PMID- 35698198 OWN - NLM STAT- MEDLINE DCOM- 20220615 LR - 20220716 IS - 1750-1172 (Electronic) IS - 1750-1172 (Linking) VI - 17 IP - 1 DP - 2022 Jun 13 TI - Analysis of 55 patients with multiple endocrine neoplasia type 1-associated insulinoma from a single center in China. PG - 219 LID - 10.1186/s13023-022-02370-1 [doi] LID - 219 AB - OBJECTIVE: To investigate the clinical characteristics of patients with multiple endocrine neoplasia type 1 (MEN1)-related insulinoma and their relationship with specific biochemical changes and to summarize the features of treatment options for the Chinese population with this disease and the impact on long-term prognosis. METHODS: "MEN1" and "insulinoma" were used when searching the Peking Union Medical College Hospital (PUMCH) medical record retrieval system to obtain clinical information about patients. We identified patients diagnosed with MEN1-associated insulinoma based on endocrinological, radiological, and pathological examinations, and subsequently analyzed their clinical data. RESULTS: A total of 55 patients with MEN1-associated insulinoma were included, including 29 (52.7%) men and 26 (47.3%) women. The parathyroid gland was the most commonly affected (78.2%), followed by the pituitary gland (69.1%) and adrenal gland (16.4%). Insulinoma was the first manifestation of MEN1 in at least 23.6% (13/55) of patients. Nineteen (34.5%) patients presented with initial symptoms of hypoglycemia before the age of 22 years. Among the 24 Patients with high serum calcium (Ca) had significantly lower serum insulin levels than those with normal serum Ca levels (p < 0.001) during hypoglycemic episodes. However, serum C-peptide level at 0.5 h and serum insulin level at 1 h was higher in patients with hypercalcemia than in patients with normal serum Ca levels in the oral glucose tolerance test (OGTT), although the differences were not statistically significant. Multifocal pancreatic neuroendocrine tumors (pNETs) were present in 38 (69.1%) patients; most of them (55.6%, 20/36) underwent multiple enucleations, and 45% (9/20) had a postoperative recurrence. Five patients (10%) who underwent distal pancreatectomy developed pancreatic insufficiency after an average of seven years. patients who underwent genetic testing, 23 (95.8%) were positive for MEN1 mutation, with mutations most commonly found in exons 2 (21.7%) and 3 (13%). CONCLUSIONS: In our study, the rates of postoperative recurrence and long-term complications in patients with MEN1 with multifocal pNETs were significantly different from those in other international centers and might be related to the choice of surgical method. In addition, elevated serum Ca levels in patients with primary hyperparathyroidism may affect insulin secretion. CI - (c) 2022. The Author(s). FAU - Zhao, Yuan AU - Zhao Y AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Yu, Jie AU - Yu J AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Liu, Yiwen AU - Liu Y AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Lyu, Lu AU - Lyu L AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Ping, Fan AU - Ping F AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Xu, Lingling AU - Xu L AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Li, Wei AU - Li W AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Wang, Ou AU - Wang O AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Xu, Qiang AU - Xu Q AD - Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Wu, Wenming AU - Wu W AD - Department of General Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. FAU - Zhang, Huabing AU - Zhang H AUID- ORCID: 0000-0001-6259-7584 AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. huabingzhangchn@163.com. AD - Chinese Academy of Medical Sciences, 1 Shuai-Fu-Yuan Wangfujing, Dongcheng District, Beijing, 100730, China. huabingzhangchn@163.com. FAU - Li, Yuxiu AU - Li Y AD - Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China. liyuxiu@medmail.com.cn. AD - Chinese Academy of Medical Sciences, 1 Shuai-Fu-Yuan Wangfujing, Dongcheng District, Beijing, 100730, China. liyuxiu@medmail.com.cn. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220613 PL - England TA - Orphanet J Rare Dis JT - Orphanet journal of rare diseases JID - 101266602 RN - 0 (Insulins) SB - IM MH - Adult MH - Female MH - Humans MH - *Hypoglycemia MH - *Insulinoma/diagnosis/pathology/surgery MH - *Insulins MH - Male MH - *Multiple Endocrine Neoplasia Type 1/genetics/pathology MH - *Neuroectodermal Tumors, Primitive MH - *Pancreatic Neoplasms/genetics/pathology MH - Young Adult PMC - PMC9195405 OTO - NOTNLM OT - Hypercalcemia OT - Hypoglycemia OT - Insulinoma OT - Multiple endocrine neoplasia type 1 COIS- The authors declare no competing interests. EDAT- 2022/06/14 06:00 MHDA- 2022/06/16 06:00 PMCR- 2022/06/13 CRDT- 2022/06/13 23:48 PHST- 2021/12/15 00:00 [received] PHST- 2022/05/29 00:00 [accepted] PHST- 2022/06/13 23:48 [entrez] PHST- 2022/06/14 06:00 [pubmed] PHST- 2022/06/16 06:00 [medline] PHST- 2022/06/13 00:00 [pmc-release] AID - 10.1186/s13023-022-02370-1 [pii] AID - 2370 [pii] AID - 10.1186/s13023-022-02370-1 [doi] PST - epublish SO - Orphanet J Rare Dis. 2022 Jun 13;17(1):219. doi: 10.1186/s13023-022-02370-1.