Familial Cancer

, Volume 15, Issue 4, pp 645–649 | Cite as

Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome

  • Tiziana Cavalli
  • Francesco Giudici
  • Raffaella Santi
  • Gabriella Nesi
  • Maria Luisa Brandi
  • Francesco Tonelli
Original Article


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.


VIPoma MEN1 Ventricular fibrillation Surgery PNET 


Author’s contribution

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.

Compliance with ethical standards

Conflict of interest

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.


  1. 1.
    Tonelli F, Giudici F, Fratini G, Brandi ML (2011) Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: review of literature. Endocr Pract 17:33–40. doi: 10.4158/EP10376.RA CrossRefPubMedGoogle Scholar
  2. 2.
    Tonelli F, Giudici F, Giusti F, Brandi ML (2012) Gastroenteropancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1. Cancers (Basel) 4:504–522. doi: 10.3390/cancers4020504 CrossRefGoogle Scholar
  3. 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
  4. 4.
    Halfdanarson TR, Rubin J, Farnell MB, Grant CS, Petersen GM (2008) Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors. Endocr Relat Cancer 15:409–427. doi: 10.1677/ERC-07-0221 CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Verner JV, Morrison AB (1958) Islet cell tumor and a syndrome of refractory watery diarrhoea and hypokalemia. Am J Med 25:374–380CrossRefPubMedGoogle Scholar
  6. 6.
    Kane MG, O’Dorisio TM, Krejs GJ (1983) Production of secretory diarrhoea by intravenous infusion of vasoactive intestinal polypeptide. N Engl J Med 309:1482–1485CrossRefPubMedGoogle Scholar
  7. 7.
    Tatemoto K, Mutt V (1980) Isolation of two novel candidate hormones using a chemical method for finding naturally occurring polypeptides. Nature 285(5764):417–418CrossRefPubMedGoogle Scholar
  8. 8.
    Blum I, Doron M, Laron Z, Atsmon A, Tiqva P (1975) Prevention of hypoglycemic attacks by propranolol in a patient suffering from insulinoma. Diabetes 24(6):535–537CrossRefPubMedGoogle Scholar
  9. 9.
    Kraft AR, Tompkins RK, Zollinger RM (1970) Recognition and management of the diarrheal syndrome caused by nonbeta islet cell tumors of the pancreas. Am J Surg 119(2):163–170CrossRefPubMedGoogle Scholar
  10. 10.
    Sninsky CA, Wolfe MM, Martin JL et al (1983) Myoelectric effects of vasoactive intestinal peptide on rabbit small intestine. Am J Physiol 244(1):G46–G51PubMedGoogle Scholar
  11. 11.
    Kulke MH, Benson AB III, Bergsland E et al (2012) Neuroendocrine tumors. J Natl Compr Cancer Netw 10(6):724–764Google Scholar
  12. 12.
    Kunz PL, Reidy-Lagunes D, Anthony LB et al (2013) Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas 42(4):557–577CrossRefPubMedPubMedCentralGoogle Scholar
  13. 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. 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. 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. 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
  17. 17.
    Peng SY, Li JT, Liu YB, Fang HQ, Wu YL, Peng CH et al (2004) Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. Pancreas 28:93–97CrossRefPubMedGoogle Scholar
  18. 18.
    Lambertini D, Bottini E, Talassi E, Tarchini R, Gaetti L, Bellomi A (2003) Acute renal failure caused by VIP-secreting tumor. G Ital Nefrol 20:419–422PubMedGoogle Scholar
  19. 19.
    Rambaud JC, Galian A, Scotto J et al (1975) Pancreatic cholera (W.D.H.A. syndrome). Histochemical and ultrastructural studies. Virchows Arch A 367(1):35–45CrossRefGoogle Scholar
  20. 20.
    Kábrt J, Dvorák M, Skrha J, Sindelka G (1999) Diagnosis and treatment of pancreatic islet cell tumors producing vasoactive intestinal polypeptide (VIPoma). Vnitr Lek 45(10):606–609PubMedGoogle Scholar
  21. 21.
    Maheshwari RR, Desai M, Rao VP, Palanki RR, Namburi RP, Reddy KT, Reddy AP (2013) Ischemic stroke as a presenting feature of VIPoma due to MEN1 syndrome. Indian J Endocrinol Metab 17:S215–S218. doi: 10.4103/2230-8210.119576 CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Thoeni RF, Mueller-Lisse UG, Chan R, Do NK, Shyn PB (2000) Detection of small, functional islet cell tumors in the pancreas: selection of MR imaging sequences for optimal sensitivity. Radiology 214:483–490CrossRefPubMedGoogle Scholar
  23. 23.
    Horton KM, Hruban RH, Yeo C, Fishman EK (2006) Multi-detector row CT of pancreatic islet cell tumors. Radiographics 26:453–464CrossRefPubMedGoogle Scholar
  24. 24.
    Semelka RC, Custodio CM, Cem Balci N, Woosley JT (2000) Neuroendocrine tumors of the pancreas: spectrum of appearances on MRI. J Magn Reson Imaging 11:141–148CrossRefPubMedGoogle Scholar
  25. 25.
    Lewis RB, Lattin GE, Paal E (2010) Pancreatic endocrine tumors: radiologic-clinicopathologic correlation. Radiographics 30:1445–1464. doi: 10.1148/rg.306105523 CrossRefPubMedGoogle Scholar
  26. 26.
    Ellison TA, Edil BH (2012) The current management of pancreatic neuroendocrine tumors. Adv Surg 46:283–296CrossRefPubMedGoogle Scholar
  27. 27.
    Anaye A, Mathieu A, Closset J, Bali MA, Metens T, Matos C (2009) Successful preoperative localization of a small pancreatic insulinoma by diffusion-weighted MRI. JOP 10:528–531PubMedGoogle Scholar
  28. 28.
    Thomason JW, Martin RS, Fincher ME (2000) Somatostatin receptor scintigraphy: the definitive technique for characterizing vasoactive intestinal peptide-secreting tumors. Clin Nucl Med 25:661–664CrossRefPubMedGoogle Scholar
  29. 29.
    Kouvaraki MA, Shapiro SE, Cote GJ, Lee JE, Yao JC, Waguespack SG, Gagel RF, Evans DB, Perrier ND (2006) Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg 30(5):643–653CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2016

Authors and Affiliations

  • Tiziana Cavalli
    • 1
  • Francesco Giudici
    • 1
  • Raffaella Santi
    • 1
  • Gabriella Nesi
    • 1
  • Maria Luisa Brandi
    • 1
  • Francesco Tonelli
    • 1
  1. 1.Department of Surgery and Translational MedicineUniversity of FlorenceFlorenceItaly

Personalised recommendations