PMID- 36889692 OWN - NLM STAT- MEDLINE DCOM- 20230630 LR - 20230630 IS - 1348-4540 (Electronic) IS - 0918-8959 (Linking) VI - 70 IP - 6 DP - 2023 Jun 28 TI - A case of vasoactive intestinal peptide-secreting tumor (VIPoma) arising from MEN1 inactivation which recurred 15 years after the initial resection. PG - 573-579 LID - 10.1507/endocrj.EJ22-0578 [doi] AB - Vasoactive intestinal peptide-secreting tumors (VIPomas) are extremely rare functional pancreatic neuroendocrine neoplasms (p-NENs) characterized by watery diarrhea, hypokalemia, and achlorhydria. Here, we report the case of a 51-year-old female patient with VIPoma that recurred after a long-term disease-free interval. This patient had been asymptomatic for approximately 15 years after the initial curative surgery for pancreatic VIPoma, with no metastasis. The patient underwent a second curative surgery for the locally recurrent VIPoma. Whole-exome sequencing of the resected tumor revealed a somatic mutation in MEN1, which is reportedly responsible not only for multiple endocrine neoplasia type 1 (MEN1) syndrome but also sporadic p-NENs. Symptoms were controlled with lanreotide before and after surgery. The patient is alive with no relapse following 14 months after surgery. This case demonstrates the importance of long-term observation of patients with VIPoma. FAU - Sakurai, Momoe AU - Sakurai M AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Wakabayashi, Tetsuji AU - Wakabayashi T AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Kondo, Yasuyuki AU - Kondo Y AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Ikeda, Eriko AU - Ikeda E AD - Department of Diagnostic Pathology, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Watanabe, Koichi AU - Watanabe K AD - Department of Pathology and Tumor Biology, Kyoto University, Kyoto 606-8501, Japan. FAU - Takei, Akihito AU - Takei A AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Okazaki, Hiroaki AU - Okazaki H AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Okada, Kenta AU - Okada K AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Ebihara, Ken AU - Ebihara K AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Kakiuchi, Nobuyuki AU - Kakiuchi N AD - The Hakubi Center for Advanced Research, Kyoto University, Kyoto 606-8501, Japan. FAU - Ogawa, Seishi AU - Ogawa S AD - Department of Pathology and Tumor Biology, Kyoto University, Kyoto 606-8501, Japan. FAU - Fukushima, Noriyoshi AU - Fukushima N AD - Department of Diagnostic Pathology, Jichi Medical University, Tochigi 329-0498, Japan. FAU - Ishibashi, Shun AU - Ishibashi S AD - Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, Jichi Medical University, Tochigi 329-0498, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20230309 PL - Japan TA - Endocr J JT - Endocrine journal JID - 9313485 RN - 37221-79-7 (Vasoactive Intestinal Peptide) SB - IM MH - Female MH - Humans MH - Middle Aged MH - *Vipoma/surgery/diagnosis/pathology MH - *Multiple Endocrine Neoplasia Type 1/complications/surgery MH - Vasoactive Intestinal Peptide MH - *Pancreatic Neoplasms/diagnosis MH - Diarrhea OTO - NOTNLM OT - Case report OT - Lanreotide OT - MEN1 OT - Pancreatic neuroendocrine neoplasm (p-NEN) OT - Vasoactive intestinal peptide-secreting tumor (VIPoma) EDAT- 2023/03/09 06:00 MHDA- 2023/06/30 06:42 CRDT- 2023/03/08 20:12 PHST- 2023/06/30 06:42 [medline] PHST- 2023/03/09 06:00 [pubmed] PHST- 2023/03/08 20:12 [entrez] AID - 10.1507/endocrj.EJ22-0578 [doi] PST - ppublish SO - Endocr J. 2023 Jun 28;70(6):573-579. doi: 10.1507/endocrj.EJ22-0578. Epub 2023 Mar 9.