PMID- 29239255 OWN - NLM STAT- MEDLINE DCOM- 20180817 LR - 20181113 IS - 1473-2300 (Electronic) IS - 0300-0605 (Print) IS - 0300-0605 (Linking) VI - 46 IP - 2 DP - 2018 Feb TI - Nonfunctional pancreatic endocrine tumor in the peripancreatic region in a Chinese patient with multiple endocrine neoplasia type 1. PG - 908-915 LID - 10.1177/0300060517728653 [doi] AB - Nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) in patients with multiple endocrine neoplasia type 1 (MEN1), which results from a mutation in the MEN1 gene, are commonly small, multiple tumors located in the pancreatic head and inside the pancreatic parenchyma. We herein describe a 35-year-old woman with bone pain and a 7-year history of a prolactinoma. She was clinically diagnosed with MEN1 based on the presence of the prolactinoma and parathyroid hyperplasia. Abdominal computed tomography revealed a 5-cm mass close to the splenic hilum. This soft tissue tumor, which was located outside the pancreatic parenchyma and the tissue origin of which could not be identified preoperatively, was found to be connected to the pancreatic tail. After resection, histological examination revealed a well-differentiated neuroendocrine tumor of pancreatic origin. Genetic testing revealed a heterozygous transition mutation of guanine to adenine at the coding nucleotide 133 in exon 2 (c.133G>A), resulting in an amino acid substitution of glutamic acid with lysine (E45K) in the MEN1 gene. This patient with MEN1 presented with a clinical condition involving a single non-metastatic NF-pNET located outside the pancreatic parenchyma with a missense mutation in the MEN1 gene, which could easily have been misdiagnosed as an accessory spleen. FAU - Wang, Bao-Ping AU - Wang BP AD - 1 Department of Endocrinology, Tianjin Medical University General Hospital, Heping District, Tianjin, China. FAU - Tian, Wei-Jun AU - Tian WJ AD - 2 Department of General Surgery, Tianjin Medical University General Hospital, Heping District, Tianjin, China. FAU - Zhang, Jie AU - Zhang J AD - 2 Department of General Surgery, Tianjin Medical University General Hospital, Heping District, Tianjin, China. FAU - Jiang, Chang-Xin AU - Jiang CX AD - 3 Department of Pathology, Tianjin Medical University General Hospital, Heping District, Tianjin, China. FAU - Qu, Hui-Qi AU - Qu HQ AD - 4 Department of Paediatrics, Division of Endocrinology, 54473 McGill University Health Centre Research Institute, Montreal, Quebec, Canada. FAU - Zhu, Mei AU - Zhu M AD - 1 Department of Endocrinology, Tianjin Medical University General Hospital, Heping District, Tianjin, China. LA - eng PT - Case Reports PT - Journal Article DEP - 20170914 PL - England TA - J Int Med Res JT - The Journal of international medical research JID - 0346411 RN - 0 (Hormone Antagonists) RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) RN - 3A64E3G5ZO (Bromocriptine) RN - Non functioning pancreatic endocrine tumor SB - IM MH - Adult MH - Amino Acid Substitution MH - Bone and Bones/physiopathology MH - Bromocriptine/therapeutic use MH - Female MH - Gene Expression MH - Hormone Antagonists/therapeutic use MH - Humans MH - Multiple Endocrine Neoplasia Type 1/diagnostic imaging/genetics/*pathology/surgery MH - *Mutation, Missense MH - Pain/diagnosis/*physiopathology MH - Pancreatic Neoplasms/diagnostic imaging/genetics/*pathology/surgery MH - Prolactinoma/diagnostic imaging/drug therapy/genetics/*pathology MH - Proto-Oncogene Proteins/*genetics MH - Tomography, X-Ray Computed PMC - PMC5971513 OTO - NOTNLM OT - MEN1 OT - NF-pNET OT - metastasis OT - missense mutation OT - pancreatic OT - parenchyma EDAT- 2017/12/15 06:00 MHDA- 2018/08/18 06:00 PMCR- 2018/02/01 CRDT- 2017/12/15 06:00 PHST- 2017/12/15 06:00 [pubmed] PHST- 2018/08/18 06:00 [medline] PHST- 2017/12/15 06:00 [entrez] PHST- 2018/02/01 00:00 [pmc-release] AID - 10.1177_0300060517728653 [pii] AID - 10.1177/0300060517728653 [doi] PST - ppublish SO - J Int Med Res. 2018 Feb;46(2):908-915. doi: 10.1177/0300060517728653. Epub 2017 Sep 14.