Medical Oncology

, Volume 19, Issue 3, pp 181–187

Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases

Dramatic and durable symptomatic benefit from hepatic artery embolization, a case report
Case Report

Abstract

Neuroendocrine tumors often manifest an excess production of hormones that create severe metabolic abnormalities resulting in significant patient morbidity, independent of the tumor burden itself. VIPomas are rare neuroendocrine tumors arising from the pancreas and are associated with secretory diarrhea and electrolyte disturbances. We present a patient with VIPoma and hepatic metastases who had greater than 10 loose stools a day for 4 yr since diagnosis, despite debulking surgery, multiple antidiarrheal medications, large doses of octreotide, and targeted radioisotope injections. The patient required several hospitalizations for treatment of dehydration and electrolyte disturbances, despite receiving daily intravenous fluids at home. Hepatic artery embolization (HAE) immediately stopped the patient’s diarrhea and provided a return to normal formed stools without any other symptom-support measures. One year after HAE, the patient remains asymptomatic and has returned to a productive life. HAE can be a very effective and durable treatment modality for patients with metastatic VIPomas (or other neuroendocrine tumors) and who are clinically symptomatic from the effects of hormone hypersecretion.

Key Words

Hepatic artery embolization VIPoma neuroendocrine tumors secretory diarrhea 

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References

  1. 1.
    Verner, J.V. and Morrison, A.B. (1958). Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am. J. Med. 9, 374–380.CrossRefGoogle Scholar
  2. 2.
    Bloom, S.R., Polak, J.M. and Pearse, A.G. (1973). Vasoactive intestinal peptide and watery-diarrhoea syndrome. Lancet 2, 14–16.PubMedCrossRefGoogle Scholar
  3. 3.
    Modlin, I.M., Lewis, J.J., Ahlman, H., Bilchik, A.J. and Kumar, R.R. (1993). Management of unresectable malignant endocrine tumors of the pancreas. Surg. Gynecol. Obstet. 176, 507–518.PubMedGoogle Scholar
  4. 4.
    Maton, P.N., Gardner, J.D. and Jensen, R.T. (1989). Use of long-acting somatostatin analog SMS 201-995 in patients with pancreatic islet cell tumors. Dig. Dis. Sci. 34, 285–395.CrossRefGoogle Scholar
  5. 5.
    Smith, S.L., et al. (1998). Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. Surgery 124, 1050–1055.PubMedCrossRefGoogle Scholar
  6. 6.
    Mekhjian, H.S. and O’Dorisio, T.M. (1987). VIPoma syndrome. Semin. Oncol. 14, 282–291.PubMedGoogle Scholar
  7. 7.
    Moertel, G.C., et al. (1994). The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann. Intern. Med. 120, 302–309.PubMedGoogle Scholar
  8. 8.
    Arcenas, A.G., Ajani, J.A., Carrasco, C.H., Levin, B. and Wallace, S. (1995). Vascular occlusive therapy of pancreatic endocrine tumors metastatic to the liver, in Endocrine Tumors of the Pancreas: Recent Advances in Research and Management (Mignon, M. and Jensen, R.T., eds), pp439–446, Karger, Basel.Google Scholar
  9. 9.
    Krausz, Y., et al. (1998). Somatostatin-receptor scintigraphy in the management of gastroenteropancreatic tumors. Am. J. Gastroenterol. 93, 66–70.PubMedCrossRefGoogle Scholar
  10. 10.
    Nguyen, H.N., et al. (1999). Long-term survival after diagnosis of hepatic metastatic VIPoma: report of two cases with disparate courses and review of therapeutic options. Dig. Dis. Sci. 44, 1148–1155.PubMedCrossRefGoogle Scholar
  11. 11.
    Yeh, C.N., Chen, M.F. and Chen, T.C. (2001). Surgical treatment of pancreatic vasoactive intestinal polypeptide-secreting tumor: a case report. Hepato-gastroenterology 48, 421–423.PubMedGoogle Scholar
  12. 12.
    Rood, R.P., DeLellis, R.A., Dayal, Y. and Donowitz, M. (1988). Pancreatic cholera syndrome due to a vasoactive intestinal polypeptide-producing tumor: further insights into the pathophysiology. Gastroenterology 94, 813–818.PubMedGoogle Scholar
  13. 13.
    Barraclough, M.A. and Bloom, S.R. (1979). Vipoma of the pancreas: observations on the diarhrhea and circulatory disturbances. Arch. Intern. Med. 139, 467–471.PubMedCrossRefGoogle Scholar
  14. 14.
    Soga, J. and Yakuwa, Y. (1998). Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J. Exp. Clin. Cancer Res. 17, 389–400.PubMedGoogle Scholar
  15. 15.
    Hammond, P.J., Gilbey, S.G., Wynick, D. and Bloom, S.R. (1993). Glucagonoma, VIPoma, somatostatinoma, other hormones, and nonfunctional tumors, in Endocrine Tumors (Mazzaferri, E. and Samaan, N.A., eds), pp457–483, Blackwell Scientific, Cambridge.Google Scholar
  16. 16.
    Brentjens, R. and Saltz, L. (2001). Islet cell tumors of the pancreas. The medical oncologist’s perspective. Surg. Clin. North Am. 81, 527–542.PubMedCrossRefGoogle Scholar
  17. 17.
    Kvols, L.K., et al. The presence of somatostatin receptors in malignant neuroendocrine tumor tissue predicts responsiveness to octreotide. Yale J. Biol. Med. 5, 505–518.Google Scholar
  18. 18.
    Lamberts, S.W., van der Lely, A.J., de Herder, W.W. and Hofland, L.J. (1996). Octreotide. N. Engl. J. Med. 334, 246–254.PubMedCrossRefGoogle Scholar
  19. 19.
    Clements, D. and Elias, E. (1985). Regression of metastatic VIPoma with somatostatin analogue SMS 201-995. [letter]. Lancet 1, 874–875.PubMedCrossRefGoogle Scholar
  20. 20.
    Kraenzlin, M.E., Ch’ng, J.L., Wood, S.M., Carr, D.H. and Bloom, S.R. (1985). Long-term treatment of a VIPoma with somatostatin analogue resulting in remission of symptoms and possible shrinkage of metastases. Gastroenterology 88, 185–187.PubMedGoogle Scholar
  21. 21.
    Venkatesh, S., Vassilopoulou-Sellin, R. and Samaan, N.A. (1989). Somatostatin analogue: use in the treatment of VIPoma with hypercalcemia. Am. J. Med. 87, 356–357.PubMedCrossRefGoogle Scholar
  22. 22.
    Gagel, R.F., et al. (1976). Streptozocin-treated Verner-Morrison syndrome: plasma vasoactive intestinal peptide and tumor responses. Arch. Intern. Med. 136, 1429–1435.PubMedCrossRefGoogle Scholar
  23. 23.
    Moertel, C.G., Lefkopoulo, M., Lipsitz, S., Hahn, R.G. and Klaassen, D. (1992). Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet cell carcinoma. N. Engl. J. Med. 326, 519–523.PubMedCrossRefGoogle Scholar
  24. 24.
    Eriksson, B. and Oberg, K. (1993) An update of the medical treatment of malignant endocrine pancreatic tumors. Acta Oncol. 32, 203–208.PubMedGoogle Scholar
  25. 25.
    Cheng, P.N. and Saltz, L.B. (1999). Failure to confirm major objective antitumor activity for streptozocin and doxorubicin in the treatment of patients with advanced islet cell carcinoma. Cancer 86, 944–948.PubMedCrossRefGoogle Scholar
  26. 26.
    Markowitz, J. (1952). The hepatic artery. Surg. Gynecol. Obstet. 95, 644–646.PubMedGoogle Scholar
  27. 27.
    Chuang, V.P. and Wallace, S. (1981). Hepatic artery embolization in the treatment of hepatic neoplasms. Radiology 140, 51–58.PubMedGoogle Scholar
  28. 28.
    Wallace, S., Charnsangavej, C., Carrasco, C.H. and Bechtel, W. (1984). Infusion embolization. Cancer 54(Suppl.), 2751–2765.PubMedCrossRefGoogle Scholar
  29. 29.
    Carrasco, C.H., Chuang, V.P. and Wallace, S. (1983). Apudomas metastatic to the liver: treatment by hepatic artery embolization. Radiology 149, 79–83.PubMedGoogle Scholar
  30. 30.
    Venook, A.P. (1999). Embolization and chemoembolization therapy for neuroendocrine tumors. Curr. Opin. Oncol. 11, 38–41.PubMedCrossRefGoogle Scholar
  31. 31.
    Marlink, R.G., Lokich, J.J., Robins, J.R. and Clouse, M.E. (1990). Hepatic arterial embolization for metastatic hormone-secreting tumors. Technique, effectiveness, and complications. Cancer 65, 2227–2232.PubMedCrossRefGoogle Scholar
  32. 32.
    Ruszniewski, P., et al. (1993). Hepatic arterial chemoembolization in patients with liver metastases of endocrine tumors. A prospective phase II study in 24 patients. Cancer 71, 2624–2630.PubMedCrossRefGoogle Scholar
  33. 33.
    Perry, L.J., Stuart, K., Stokes, K.R. and Clouse, M.E. (1994). Hepatic arterial chemoembolization for metastatic neuroendocrine tumors. Surgery 116, 1111–1116.PubMedGoogle Scholar
  34. 34.
    Ajani, J.A., et al. (1988). Islet cell tumors metastatic to the liver: effective palliation by sequential hepatic artery embolization. Ann. Intern. Med. 108, 340–344.PubMedGoogle Scholar
  35. 35.
    Carrasco, C.H., et al. (1986). The carcinoid syndrome: palliation by hepatic artery embolization. Am. J. Roentgenol. 147, 149–154.Google Scholar
  36. 36.
    Drougas, J.G., et al. (1998). Hepatic artery chemoembolization for management of patients with advanced metastatic carcinoid tumors. Am. J. Surg. 175, 408–412.PubMedCrossRefGoogle Scholar
  37. 37.
    Bierman, H.R., Byron, R.L. and Kelly, R.H. (1951). Studies on the blood supply of tumors in man: arteriography in vivo. J. Natl. Cancer Inst. 12, 107–131.PubMedGoogle Scholar
  38. 38.
    Breedis, C. and Young, G. (1954). The blood supply of neoplasms in the liver. Am. J. Pathol. 30, 969–985.PubMedGoogle Scholar
  39. 39.
    Chuang, V.P., Wallace, S., Soo, C.S., Charnsangavej, C. and Bowers, T. (1982). Therapeutic ivalon embolization of hepatic tumors. Am. J. Roentgenol. 138, 289–294.Google Scholar
  40. 40.
    Soo, C.S., Chuang, V.P., Wallace, S., Charnsangavej, C. and Carrasco, H. (1983). Treatment of hepatic neoplasm through extrahepatic collaterals. Radiology 147, 45–49.PubMedGoogle Scholar
  41. 41.
    Vassilopoulou-Sellin, R. and Ajani, J. (1994). Islet cell tumors of the pancreas. Endocrinol. Metab. Clin. North Am. 23, 53–65.PubMedGoogle Scholar

Copyright information

© Humana Press Inc 2002

Authors and Affiliations

  • C. C. Case
    • 1
  • K. Wirfel
    • 2
  • R. Vassilopoulou-Sellin
    • 2
  1. 1.Division of Endocrinology and MetabolismBaylor College of MedicineHouston
  2. 2.Department of Endocrine Neoplasia and Hormonal DisordersThe University of Texas M.D. Anderson Cancer CenterHouston

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