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*[[Sandostatin|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) | *[[Sandostatin|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).<ref name="pmid2545444">{{cite journal| author=O'Dorisio TM, Mekhjian HS, Gaginella TS| title=Medical therapy of VIPomas. | journal=Endocrinol Metab Clin North Am | year= 1989 | volume= 18 | issue= 2 | pages= 545-56 | pmid=2545444 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2545444 }}</ref> | *[[Steroids]] are used in diarrhea of VIPoma refractory to somatostatin (prednisone 60 mg per day).<ref name="pmid2545444">{{cite journal| author=O'Dorisio TM, Mekhjian HS, Gaginella TS| title=Medical therapy of VIPomas. | journal=Endocrinol Metab Clin North Am | year= 1989 | volume= 18 | issue= 2 | pages= 545-56 | pmid=2545444 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2545444 }}</ref> | ||
*Sunitinib a tyrosin kinase inhibitor has some evidence of symptomatic and biochemical control in somatostatin analogue resistant VIPoma.<ref name="pmid27461388">{{cite journal| author=Dimitriadis GK, Weickert MO, Randeva HS, Kaltsas G, Grossman A| title=Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours. | journal=Endocr Relat Cancer | year= 2016 | volume= 23 | issue= 9 | pages= R423-36 | pmid=27461388 | doi=10.1530/ERC-16-0200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27461388 }}</ref><ref name="pmid23840053" /> It is also a medical treatment for locally advanced unresectable or advanced metastatic VIPoma. | *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.<ref name="pmid27461388">{{cite journal| author=Dimitriadis GK, Weickert MO, Randeva HS, Kaltsas G, Grossman A| title=Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours. | journal=Endocr Relat Cancer | year= 2016 | volume= 23 | issue= 9 | pages= R423-36 | pmid=27461388 | doi=10.1530/ERC-16-0200 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27461388 }}</ref><ref name="pmid23840053" /> It is also a medical treatment for locally advanced unresectable or advanced metastatic VIPoma. | ||
=Medical management of advanced local or metastatic disease= | =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. | *There has been limited use of chemotherapy in patients with VIPoma. However, streptozocin based chemotherapy is considered best. | ||
*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 for concerns of adverse effects but is inferior.<ref name="pmid15570077">{{cite journal| author=Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R et al.| title=Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. | journal=J Clin Oncol | year= 2004 | volume= 22 | issue= 23 | pages= 4762-71 | pmid=15570077 | doi=10.1200/JCO.2004.04.024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15570077 }}</ref> | *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 for concerns of adverse effects but is inferior.<ref name="pmid15570077">{{cite journal| author=Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R et al.| title=Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. | journal=J Clin Oncol | year= 2004 | volume= 22 | issue= 23 | pages= 4762-71 | pmid=15570077 | doi=10.1200/JCO.2004.04.024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15570077 }}</ref> | ||
*Molecularly targeted therapy eg. Sunitinib (tyrosine kinase inhibitor) is approved for treatment of progressive, unresectable, locally advanced or metastatic well differenctiated VIPoma. It is used in combination with somatostatin analogue.<ref name="pmid23840053">{{cite journal| author=Zhang J, Francois R, Iyer R, Seshadri M, Zajac-Kaye M, Hochwald SN| title=Current understanding of the molecular biology of pancreatic neuroendocrine tumors. | journal=J Natl Cancer Inst | year= 2013 | volume= 105 | issue= 14 | pages= 1005-17 | pmid=23840053 | doi=10.1093/jnci/djt135 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23840053 }}</ref> | *Molecularly targeted therapy eg. Sunitinib [37.5 mg per day to maximum 50 mg per day] (a tyrosine kinase inhibitor) is approved for treatment of progressive, unresectable, locally advanced or metastatic well differenctiated VIPoma. It is used in combination with somatostatin analogue.<ref name="pmid23840053">{{cite journal| author=Zhang J, Francois R, Iyer R, Seshadri M, Zajac-Kaye M, Hochwald SN| title=Current understanding of the molecular biology of pancreatic neuroendocrine tumors. | journal=J Natl Cancer Inst | year= 2013 | volume= 105 | issue= 14 | pages= 1005-17 | pmid=23840053 | doi=10.1093/jnci/djt135 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23840053 }}</ref> | ||
*Other molecular targeted therapy undergoing research for treatment are Everolimus (mTOR inhibitor) and Bevacizumab ( anti-VEGF monoclonal antibody). | *Other molecular targeted therapy undergoing research for treatment are Everolimus (mTOR inhibitor) and Bevacizumab ( anti-VEGF monoclonal antibody).<ref name="pmid1310159">{{cite journal| author=Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D| title=Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. | journal=N Engl J Med | year= 1992 | volume= 326 | issue= 8 | pages= 519-23 | pmid=1310159 | doi=10.1056/NEJM199202203260804 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1310159 }}</ref> | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 15:16, 9 January 2018
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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.
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.
- 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 for concerns of adverse effects but is inferior.[5]
- Molecularly targeted therapy eg. Sunitinib [37.5 mg per day to maximum 50 mg per day] (a tyrosine kinase inhibitor) is approved for treatment of progressive, unresectable, locally advanced or metastatic well differenctiated VIPoma. It is used in combination with somatostatin analogue.[4]
- 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.
- ↑ 4.0 4.1 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.