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. Somatostatin analogues like short acting octreotide is useful for controlling diarrhea by blocking the release of VIP. Octreotide is later replaced by longer acting depot preparation of somatostatin analogues like sandostatin or lanreotide.
Medical Therapy
Initial 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.
Medical management
- Somatostatin analogues like short acting octreotide is useful for controlling diarrhea by blocking the release of VIP.
- Octreotide is later replaced by longer acting depot preparation of somatostatin analogues like sandostatin or lanreotide.
- Steroids are used in diarrhea of VIPoma refractory to somatostatin.[1]
- 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.[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]
- Other molecular targeted therapy undergoing research for treatment are Everolimus (mTOR inhibitor) and Bevacizumab ( anti-VEGF monoclonal antibody).[5]
References
- ↑ O'Dorisio TM, Mekhjian HS, Gaginella TS (1989). "Medical therapy of VIPomas". Endocrinol Metab Clin North Am. 18 (2): 545–56. PMID 2545444.
- ↑ 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.
- ↑ 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.
- ↑ 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.