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

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

Abstract

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.

Keywords: Case report; Lanreotide; MEN1; Pancreatic neuroendocrine neoplasm (p-NEN); Vasoactive intestinal peptide-secreting tumor (VIPoma).

Publication types

  • Case Reports

MeSH terms

  • Diarrhea
  • Female
  • Humans
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1* / complications
  • Multiple Endocrine Neoplasia Type 1* / surgery
  • Pancreatic Neoplasms* / diagnosis
  • Vasoactive Intestinal Peptide
  • Vipoma* / diagnosis
  • Vipoma* / pathology
  • Vipoma* / surgery

Substances

  • Vasoactive Intestinal Peptide