VIPoma Presenting with Obstructive Jaundice
A Case Report
DOI:
https://doi.org/10.15605/jafes.041.02.6165Keywords:
VIPoma, diarrhea, pancreatic neuroendocrine tumorAbstract
VIPoma is a rare pancreatic neuroendocrine tumor characterized by the overproduction of vasoactive intestinal peptide (VIP), typically manifesting as watery diarrhea, hypokalaemia and metabolic acidosis. Its presentation can be atypical, complicating diagnosis. We report the case of a 30-year-old male who initially presented with obstructive jaundice and fatigue, with imaging revealing a pancreatic head mass and liver metastases. Histopathological analysis confirmed a low-grade pancreatic neuroendocrine tumor. The clinical course was complicated by portal vein thrombosis and metastatic spread. Six months later, the patient developed persistent, refractory watery diarrhea with associated hypokalaemia. Elevated serum VIP levels and Gallium-68 DOTATATE positron-emission tomography and computed tomography (PET-CT) supported the diagnosis of VIPoma. Symptom resolution was achieved with subcutaneous octreotide, followed by transition to monthly long-acting release (LAR) octreotide. Approximately 60% of VIPoma cases are metastatic at the time of diagnosis, as demonstrated in this patient. The case highlighted the diagnostic challenges of VIPoma in the presence of atypical symptoms such as jaundice and absence of initial diarrhea. It also underscores the need for close multidisciplinary collaboration to determine the best course of action and to optimize patient outcomes.
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References
1. Karele EN. All you need to know about VIPoma: Review on the latest studies. Presse Med. 2024;53(2):104222. https://pubmed.ncbi.nlm.nih.gov/38109967 https://doi.org/10.1016/j.lpm.2023.104222
2. Basuroy R, Bouvier C, Ramage J, Sissons M, Srirajaskanthan R. Delays and routes to diagnosis of neuroendocrine tumors. BMC Cancer. 2018;18(1):1122. https://pubmed.ncbi.nlm.nih.gov/30445941https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240263 https://doi.org/10.1186/s12885-018-5057-3
3. Schizas D, Mastoraki A, Bagias G, et al. Clinicopathological data and treatment modalities for pancreatic VIPomas: A systematic review. J BUON. 2019;24(2):415-23. https://pubmed.ncbi.nlm.nih.gov/31127985
4. Azizian A, König A, Ghadimi M. Treatment options of metastatic and nonmetastatic VIPoma: a review. Langenbecks Arch Surg. 2022;407(7):2629-36. https://pubmed.ncbi.nlm.nih.gov/35931878 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9640398 https://doi.org/10.1007/s00423-022-02620-7
5. Helbing A, Menon G, Sandhu S, et al. VIPoma. In: StatPearls. StatPearls Publishing; Updated February 23, 2025. Bookshelf ID: NBK507698
6. Boccatonda A, Gentilini S, Zanata E, Simion C, Serra C, Simioni P, et al. Portal vein thrombosis: State-of-the-art review. J Clin Med. 2024;13(5):1517. https://pubmed.ncbi.nlm.nih.gov/38592411 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10932352 https://doi.org/10.3390/jcm13051517
7. Bicci E, Cozzi D, Ferrari R, Grazzini G, Pradella S, Miele V. Pancreatic neuroendocrine tumors: Spectrum of imaging findings. Gland Surg. 2020;9(6):2215-24. https://pubmed.ncbi.nlm.nih.gov/33447574 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7804535 https://doi.org/10.21037/gs-20-537
8. Park S, Parihar AS, Bodei L, Hope TA, Mallak N, Millo C, et al. Somatostatin receptor imaging and theranostics: Current practice and future prospects. J Nucl Med. 2021;62(10):1323-29. https://pubmed.ncbi.nlm.nih.gov/34301785 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9364764 https://doi.org/10.2967/jnumed.120.251512
9. Brugel M, Walter T, Goichot B, et al. Efficacy of treatments for VIPoma: A GTE multicentric series. Pancreatology. 2021;21(8):1531-9. https://pubmed.ncbi.nlm.nih.gov/34404601 https://doi.org/10.1016/j.pan.2021.08.001
10. de Herder WW, Hofland J. Vasoactive intestinal peptide-secreting tumor (VIPoma). In: Feingold KR, Adler RA, Ahmed SF, et al., eds. Endotext. South Dartmouth (MA): MDText.com, Inc.; 2000–. Bookshelf ID: NBK278960/
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