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__NOTOC__
__NOTOC__
{{VIPoma}}
{{VIPoma}}
{{CMG}}{{AE}}{{MSI}}{{PSD}}
{{CMG}}{{AE}}{{MSI}}{{PSD}}{{Homa}}
==Overview==
==Overview==
Initial treatment in patient with VIPoma is prompt replacement of fluid and correction of electrolyte imbalance and acid-base disturbance. [[Steroids]] may be used to provide symptomatic relief.<ref name="sp">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/</ref>
Initial treatment in [[patient]] with VIPoma is [[prompt]] [[Fluid replacement therapy|replacement of fluid]] and correction of [[electrolyte imbalance]] and [[Acid-base disturbances|acid-base disturbance]]. [[Sandostatin|Somatostatin]] analogues like short acting [[octreotide]] is useful for controlling [[diarrhea]] by blocking the release of [[Vasoactive intestinal peptide|VIP]]. [[Octreotide]] is later replaced by longer acting depot preparation of [[somatostatin]] analogues like [[sandostatin]] or [[lanreotide]].


==Medical Therapy==
==Medical Therapy==
*Initial treatment in patient with VIPoma is prompt replacement of fluid and electrolyte losses. The IV fluid of choice is isotonic normal saline.
===Initial treatment===
*[[Sandostatin]] or [[chemotherapy]] must be considered for malignant tumors.
*Initial treatment in patient with VIPoma is [[prompt]] [[Fluid replacement therapy|replacement of fluid]] and [[Electrolyte disturbance|electrolyte losses]].
*[[Steroids]] may be used to provide symptomatic relief.
*The [[IV fluids|IV fluid]] of choice is [[isotonic]] [[normal saline]] with added [[potassium]] and [[bicarbonate]] as necessary.
*Prostaglandin synthesis inhibitors (e.g., [[indomethacin]]), [[phenothiazines]], and [[lithium]] combination may be used.
*Long-term [[octreotide]] treatment not only controls the [[diarrhea]] in the patients with VIPoma but also may cause arrest or regression of the tumor.


=== Medical management ===
*[[Sandostatin|Somatostatin]] analogues like short acting [[octreotide]] is useful for controlling [[diarrhea]] by blocking the release of [[Vasoactive intestinal peptide|VIP]].<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>
*[[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]].
*'''<u>Initial Management</u>'''
** '''Preferred regimen (1)''' : [[Octreotide]] 50-100mcg q8h initially
* '''<u>Maintenance dosage</u>'''
**'''Preferred regimen (1)''': [[Sandostatin]] 20 mg IM every 4 weeks [[Lanreotide]] (120mg [[Subcutaneous|subcutaneously]] every 4 weeks). '''(OR)'''
** '''Preferred regimen (2)''': [[Lanreotide]] 120mg  [[Subcutaneous|subcutaneously]] every 4 weeks.
* '''<u>Refractory cases</u>'''
** '''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]].<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><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><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">{{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>
*Most successful and favourable combination regimen of [[chemotherapy]] is [[streptozocin]] with [[Doxorubicin hydrochloride|doxorubicin]] together with [[somatostatin]] analogues.
*[[Fluorouracil|5-FU]] can be used as alternative to [[Doxorubicin hydrochloride|doxorubicin]] in [[patients]] with who have [[contraindications]] to [[Doxorubicin|doxorubucin]].
*[[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.
*Other [[molecular]] [[targeted therapy]] undergoing [[research]] for treatment are [[Everolimus]] ([[mTOR]] [[inhibitor]]) and [[Bevacizumab]] ( anti-[[Vascular endothelial growth factor|VEGF]] [[monoclonal antibody]]).
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 00:40, 30 July 2020

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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] Homa Najafi, M.D.[4]

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

Medical management

Medical management of advanced local or metastatic disease

References

  1. O'Dorisio TM, Mekhjian HS, Gaginella TS (1989). "Medical therapy of VIPomas". Endocrinol Metab Clin North Am. 18 (2): 545–56. PMID 2545444.
  2. 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.
  3. 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.
  4. 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.
  5. 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.


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