Published Date: March 8, 2023

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A case of vasoactive intestinal peptide-secreting tumor (VIPoma) arising from MEN1 inactivation which recurred 15 years after the initial resection

Authors: Momoe Sakurai, Tetsuji Wakabayashi, Yasuyuki Kondo, Eriko Ikeda, Koichi Watanabe, Akihito Takei, Hiroaki Okazaki, Kenta Okada, Ken Ebihara, Nobuyuki Kakiuchi, Seishi Ogawa, Noriyoshi Fukushima, Shun Ishibashi

Endocr J. 2023 Jun 28;70(6):573-579. doi: 10.1507/endocrj.EJ22-0578. Epub 2023 Mar 9.


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.

PMID: 36889692DOI: 10.1507/endocrj.EJ22-0578