VIPoma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Madhu Sigdel M.B.B.S.[2]Parminder Dhingra, M.D. [3]
Overview
Initial treatment in patient with VIPoma is prompt replacement of fluid and correction of electrolyte imbalance and acid-base disturbance.[1]
Medical Therapy
Symptomatic treatment
- Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte losses.
- The IV fluid of choice is isotonic normal saline with added potassium and bicarbonate as necessary.
- Somatostatin analogues like short acting octreotide (50-100mcg every 8 hours) are useful for controlling diarrhea by blocking the release of VIP.
- Octreotide is later replaced by longer acting depot preparation of somatostatin analogues like sandostatin (20 mg IM every 4 weeks) and lanreotide (120mg subQ every 4 weeks).
- Steroids are used in diarrhea of VIPoma refractory to somatostatin (prednisone 60 mg per day).[2]
- Sunitinib (37.5 mg oral once a day) a tyrosin kinase inhibitor has some evidence of symptomatic and biochemical control in somatostatin analogue resistant VIPoma.[3][4] It is also a medical treatment for locally advanced unresectable or advanced metastatic VIPoma.
- Initial Management
- Preferred regimen (1) : Octreotide 50-100mcg q8h initially
- Maintenance dosage
- Preferred regimen (1): Sandostatin 20 mg IM every 4 weeks Lanreotide (120mg subQ every 4 weeks). (OR)
- Preferred regimen (2): Lanreotide 120mg subcutaneously every 4 weeks.
- Refractory cases
- Preferred regimen (1): Prednisone 60 mg IM q24 for 1 week.
- Preferred regimen (2): Sunitinib 37.5 mg PO q24h.
Medical management of advanced local or metastatic disease
- There has been limited use of chemotherapy in patients with VIPoma. However, streptozocin based chemotherapy is considered best in the management of advanced local or metastatic disease.
- Most successful and favourable combination regimen of chemotherapy is streptozocin with doxorubicin together with somatostatin analogues.
- 5-FU can be used as alternative to doxorubicin in patients with who have contraindications to doxorubucin.[5]
- Other molecular targeted therapy undergoing research for treatment are Everolimus (mTOR inhibitor) and Bevacizumab ( anti-VEGF monoclonal antibody).[6]
References
- ↑ Vinik A. Vasoactive Intestinal Peptide Tumor (VIPoma) [Updated 2013 Nov 28]. In: De Groot LJ, Beck-Peccoz P, Chrousos G, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: http://www.ncbi.nlm.nih.gov/books/NBK278960/
- ↑ O'Dorisio TM, Mekhjian HS, Gaginella TS (1989). "Medical therapy of VIPomas". Endocrinol Metab Clin North Am. 18 (2): 545–56. PMID 2545444.
- ↑ Dimitriadis GK, Weickert MO, Randeva HS, Kaltsas G, Grossman A (2016). "Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours". Endocr Relat Cancer. 23 (9): R423–36. doi:10.1530/ERC-16-0200. PMID 27461388.
- ↑ Zhang J, Francois R, Iyer R, Seshadri M, Zajac-Kaye M, Hochwald SN (2013). "Current understanding of the molecular biology of pancreatic neuroendocrine tumors". J Natl Cancer Inst. 105 (14): 1005–17. doi:10.1093/jnci/djt135. PMID 23840053.
- ↑ Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R; et al. (2004). "Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas". J Clin Oncol. 22 (23): 4762–71. doi:10.1200/JCO.2004.04.024. PMID 15570077.
- ↑ Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D (1992). "Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma". N Engl J Med. 326 (8): 519–23. doi:10.1056/NEJM199202203260804. PMID 1310159.