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Case Reports
. 2016 Oct;101(10):3564-3567.
doi: 10.1210/jc.2016-2051. Epub 2016 Sep 1.

Pancreatic Neuroendocrine Tumor Secreting Vasoactive Intestinal Peptide and Dopamine With Pulmonary Emboli: A Case Report

Naris Nilubol  1 , Esther M Freedman  1 , Martha M Quezado  1 , Dhaval Patel  1 , Electron Kebebew  1
Affiliations
Case Reports

Pancreatic Neuroendocrine Tumor Secreting Vasoactive Intestinal Peptide and Dopamine With Pulmonary Emboli: A Case Report

Naris Nilubol et al. J Clin Endocrinol Metab. 2016 Oct.

Abstract

Context: The vasoactive intestinal peptide-secreting neuroendocrine tumor (VIPoma) is a very rare pancreatic tumor. We report the first case of a patient with VIPoma that co-secreted dopamine and had pulmonary emboli.

Case description: A 67-year-old woman presented with 2 months of watery diarrhea, severe generalized weakness,6.8 kg of weight loss, a facial rash, and hypokalemia. Colonoscopy did not reveal the cause of the chronic diarrhea. Initial biochemical testing showed markedly elevated serum vasoactive intestinal peptide (VIP) and pancreatic polypeptide. Computed tomography scan of the abdomen and pelvis revealed a 5.4-cm distal pancreatic mass. Octreoscan showed an intense uptake in the area of the pancreatic mass. Incidental pulmonary emboli were found and treated. Additional biochemical testing revealed a markedly elevated urinary dopamine level. The patient received preoperative α-blockade and octreotide. She underwent a successful laparoscopic distal pancreatectomy. Postoperative urinary dopamine and pancreatic polypeptide were within normal limits. Serum VIP decreased by half but remained elevated. Pathology confirmed a grade 1 pancreatic neuroendocrine tumor without lymph node metastasis. The patient's symptoms resolved and no longer required octreotide. Metastatic workup including computed tomography, F18-fluorodeoxglucose positron emission tomography, and Ga68-DOTATATE scans were negative during 4 years of follow-up.

Conclusions: VIPoma is a rare subtype of pancreatic neuroendocrine tumor that can secrete dopamine and can be associated with thromboembolism.

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Figures

Figure 1.
Figure 1.
Radiographic imaging of a patient with VIPoma. A, Contrast-enhanced CT scan of the abdomen showed pancreatic tail mass (blue arrow). B, A pulmonary CT angiography showed right segmental pulmonary artery-filling defects. C, Octreoscan. D, F18-FDG PET/CT scan showed an intense uptake in the pancreatic tail mass.
Figure 2.
Figure 2.
Histology of PNET secreting VIP and dopamine. A, Hematoxylin and eosin staining; B, synaptophysin; C, chromogranin; and D, pancreatic polypeptide immunohistochemistry.
Figure 3.
Figure 3.
VIP expression in the adjacent normal pancreas (A) and in the PNET (B). DOPA-decarboxylase expression in the adjacent normal pancreas (C) and in the PNET (D).
See this image and copyright information in PMC

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