Surgical treatment of metastatic VIPoma: a case report

VIPoma, a neuroendocrine tumour mostly occurring in the human pancreas and producing high levels of vasoactive intestinal peptide, is a rare disease that presents with a wide spectrum of symptoms, including intense diarrhoea, hypokalaemia, and cardiac complications, with life-threatening consequences. In most cases, metastatic lesions are present at VIPoma diagnosis. Treatment options include symptomatic therapy, chemotherapy, radiation and surgery. Due to its low incidence, there are no evidence-based therapy recommendations to date. Here, we present a case of a 39-year-old woman with severe symptoms due to VIPoma of the pancreas with diffuse hepatic metastasis, who underwent simultaneous resection of the primary tumour, extensive liver resection and radiofrequency ablation. The patient was released in good health and was recurrence-free during 12 months surveillance. According to the existing literature and our own experience, surgical procedures appear to be the most promising therapy option for cases with diffuse hepatic metastasis, offering patients relief from their symptoms and (chemo)therapy-free time.

1. Gardner, JD, Cerda, JJ. In vitro inhibition of intestinal fluid and electrolyte transfer by a non-beta islet cell tumor. Proc Soc Exp Biol Med 1966; 123: 361–364.
Google Scholar | SAGE Journals2. Iwasaki, M, Akiba, Y, Kaunitz, JD. Recent advances in vasoactive intestinal peptide physiology and pathophysiology: focus on the gastrointestinal system. F1000Res 2019; 8: 1629.
Google Scholar | Crossref3. Verner, JV, Morrison, AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med 1958; 25: 374–380.
Google Scholar | Crossref | Medline | ISI4. Riley, DS, Barber, MS, Kienle, GS, et al. CARE guidelines for case reports: Explanation and elaboration document. J Clin Epidemiol. 2017; 89: 218–235.
Google Scholar | Crossref | Medline5. Rindi, G, Kloppel, G, Alhman, H et al. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2006; 449: 395–401.
Google Scholar6. Perry, RR, Vinik, AI. Clinical review 72: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab 1995; 80: 2273–2278.
Google Scholar | Medline | ISI7. Smith, SL, Branton, SA, Avino, AJ, et al. Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. Surgery 1998; 124: 1050–1055.
Google Scholar | Crossref | Medline8. Bourcier, ME, Vinik, AI. Sunitinib for the treatment of metastatic paraganglioma and vasoactive intestinal polypeptide-producing tumor (VIPoma). Pancreas 2013; 42: 348–352.
Google Scholar | Crossref | Medline | ISI9. Amiri, FS. Prevalence of diagnostic methods and treatment modalities in vipoma patients: a rare cause of hormone-mediated diarrhea. Indian J Endocrinol Metab 2019; 23: 318–325.
Google Scholar | Crossref | Medline10. Marques, B, Monteiro, AR, Martins, RG, et al. Metastatic VIPoma, cosecreting insulin, with complete response to lanreotide, capecitabine, and temozolomide. Pancreas 2020; 49: e19–e20.
Google Scholar | Crossref | Medline11. Sandhu, S, Jialal, I. ViPoma. Treasure Island, FL: StatPearls, 2020.
Google Scholar12. Ueda, Y, Toyama, H, Terai, S, et al. [A surgical resected case of vipoma with para-aortic lymph node involvement]. Gan To Kagaku Ryoho 2017; 44: 1976–1978.
Google Scholar | Medline13. Abu-Zaid, A, Azzam, A, Abudan, Z, et al. Sporadic pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) in a 47-year-old male. Hematol Oncol Stem Cell Ther 2014; 7: 109–115.
Google Scholar | Crossref | Medline

留言 (0)

沒有登入
gif