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Neuroendocrine tumors with syndromic vasoactive intestinal polypeptide hypersecretion: a retrospective study
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Tumor Biology.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical and experimental pathology.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Radiology.
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2017 (English)In: International Journal of Endocrine Oncology, Vol. 4, no 1, p. 9-22Article in journal (Refereed) Published
Abstract [en]

Aim: Vasoactive intestinal polypeptide producing neuroendocrine tumors are rare and cause severe hormonal symptoms. Patients/methods: Eighteen patients with vasoactive intestinal polypeptide producing neuroendocrine tumors were analyzed with reviews of medical records, radiology and tumor tissue specimens. Results: Twelve patients (67%) had liver metastases at diagnosis. Chemotherapy, somatostatin analogs and interferon were given as medical therapies. Streptozocin/5-fluorouracil produced an objective response in 40% of the evaluable patients. Somatostatin analogs gave a clinical/biochemical response in eight out of nine patients. Transarterial embolization of the liver and peptide receptor radionuclide therapy was given to refractory cases. Sixteen patients died during the observation period. The median overall survival from diagnosis was 102 months. Conclusion: Systemic chemotherapy and somatostatin analogs should be given in cases of advanced disease or for hormonal symptoms.

Place, publisher, year, edition, pages
2017. Vol. 4, no 1, p. 9-22
Keywords [en]
chemotherapy, IFN-α, neuroendocrine tumors, pancreas, somatostatin analogs, survival, vasoactive intestinal polypeptide
National Category
Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-342674DOI: 10.2217/ije-2016-0012ISI: 000426222300003OAI: oai:DiVA.org:uu-342674DiVA, id: diva2:1184997
Available from: 2018-02-22 Created: 2018-02-22 Last updated: 2018-03-23Bibliographically approved

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Publisher's full texthttps://www.futuremedicine.com/doi/abs/10.2217/ije-2016-0012

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Antonodimitrakis, Pantelis ClewemarGrimelius, LarsSundin, AndersWassberg, CeciliaGranberg, DanSkogseid, BrittEriksson, Barbro

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Endocrine Tumor BiologyClinical and experimental pathologyExperimental and Clinical OncologyRadiologyEndocrin Oncology
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