Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome
Sporadic VIPoma is an exceedingly rare tumor with an annual incidence of 1:10 million people worldwide, yet it is described in approximately 5 % of MEN1 patients. The majority of VIPomas are malignant and radical surgery is the best therapeutic option. A 58-year-old man presented with cardiocirculatory arrest due to ventricular fibrillation. The patient had a 3-month history of epigastric pain with diarrhea. After reanimation, laboratory data revealed severe hypokalemia and hypercalcemia. Further investigations showed hyperparathyroidism, left adrenal adenoma and pituitary microprolactinoma and genetic diagnosis of MEN1 syndrome was made. Abdominal computed tomography revealed a 45 × 30 mm mass of the pancreatic head and two hepatic lesions, which proved to be neuroendocrine after 68 Ga PET and needle biopsy. Vasoactive intestinal peptide (VIP) serum level had increased. Subsequently the patient underwent pylorus-preserving pancreaticoduodenectomy and hepatic resection. Intraoperative VIP returned to normal values. Histopathology confirmed a pancreatic VIPoma metastatic to the liver. The postoperative course was unremarkable and the patient is well with no evidence of disease at a 48 months follow-up. Even in case of anusual presentation, when two or more main clinical findings of MEN1 related tumors are present, unrespectively to the presence of MEN1 mutation, MEN1 syndrome should be suspected. Surgery in MEN1 pancreatic neuroendocrine tumors is indicated both to treat symptoms and to avoid oncological progression even in advanced cases.
KeywordsVIPoma MEN1 Ventricular fibrillation Surgery PNET
T. C. and F. G. collected the data; T. C., F. G. and G. N. analyzed data; T. C., F. G., F. T. wrote the manuscript; G. N. and M. L. B. supervised the manuscript and T. C., F. G., G. N., F. T. and M. L. B. revised the manuscript.
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The authors certify that there is no actual or potential conflict of interest in relation to this article and they state that there are no financial interests or connections, direct or indirect, or other situations that might raise the question of bias in the work reported or the conclusions, implications, or opinions stated—including pertinent commercial or other sources of funding for the individual author(s) or for the associated department(s) or organization(s), personal relationships, or direct academic competition.
- 3.Goudet P, Murat A, Binquet C, Cardot-Bauters C, Costa A, Ruszniewski P, Niccoli P, Ménégaux F, Chabrier G, Borson-Chazot F, Tabarin A, Bouchard P, Delemer B, Beckers A, Bonithon-Kopp C (2010) Risk factors and causes of death in MEN1 disease. A GTE (Groupe d’Etude des Tumeurs Endocrines) cohort study among 758 patients. World J Surg 34(2):249–255. doi: 10.1007/s00268-009-0290-1 CrossRefPubMedGoogle Scholar
- 11.Kulke MH, Benson AB III, Bergsland E et al (2012) Neuroendocrine tumors. J Natl Compr Cancer Netw 10(6):724–764Google Scholar
- 13.Triponez F, Dosseh D, Goudet P, Cougard P, Bauters C, Murat A, Cadiot G, Niccoli-Sire P, Chayvialle JA, Calender A, Proye CA (2006) Epidemiology data on 108 MEN 1 patients from the GTE with isolated nonfunctioning tumors of the pancreas. Ann Surg 243(2):265–272CrossRefPubMedPubMedCentralGoogle Scholar
- 14.Sakurai A, Suzuki S, Kosugi S, Okamoto T, Uchino S, Miya A, Imai T, Kaji H, Komoto I, Miura D, Yamada M, Uruno T, Horiuchi K, Miyauchi A, Imamura M, MEN Consortium of Japan, Fukushima T, Hanazaki K, Hirakawa S, Igarashi T, Iwatani T, Kammori M, Katabami T, Katai M, Kikumori T, Kiribayashi K, Koizumi S, Midorikawa S, Miyabe R, Munekage T, Ozawa A, Shimizu K, Sugitani I, Takeyama H, Yamazaki M (2012) Multiple endocrine neoplasia type 1 in Japan: establishment and analysis of a multicentre database. Clin Endocrinol (Oxf) 76(4):533–539. doi: 10.1111/j.1365-2265.2011.04227.x CrossRefGoogle Scholar
- 15.Jensen RT, Norton JA (2002) Pancreatic endocrine tumors. In: Felman M, Friedman LS, Sleisenger MH (eds) Sleisenger and Fordtran’s gastrointestinal and liver diseases. Saunders, Philadelphia, pp 988–1016Google Scholar
- 16.Hasegawa T, Yamao K, Hijioka S, Bhatia V, Mizuno N, Hara K, Imaoka H, Niwa Y, Tajika M, Kondo S, Tanaka T, Shimizu Y, Kinoshita T, Kohsaki T, Nishimori I, Iwasaki S, Saibara T, Hosoda W, Yatabe Y (2014) Evaluation of Ki-67 index in EUS–FNA specimens for the assessment of malignancy risk in pancreatic neuroendocrine tumors. Endoscopy 46(1):32–38. doi: 10.1055/s-0033-1344958 (Epub 11 Nov 2013) PubMedGoogle Scholar