Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 12, Number 5, May 2021, pages 195-201

Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature


Figure 1.
Figure 1. Clinical classification of neuroendocrine tumors (NETs).
Figure 2.
Figure 2. Pancreatic VIPoma.
Figure 3.
Figure 3. (a) Octreotide scintigraphy showing hyperintense lesion pancreas. (b) Magnetic simple and proven abdomen showing focal lesions solid appearance adjacent to the posterior and superior aspect of the pancreas body (arrow).


Table 1. VIPoma Classification
2017 WHO panNEN Tumor ClassificationKi proliferation index (%)*Mitotic index
*The Ki-67 proliferation index is based on the evaluation of ≥ 500 cell in areas of higher nuclear labeling (hot spot). The mitotic index is based on the evaluation of mitoses in 50 high-power fields in areas of higher density, and is expressed as mitoses per 10 high-power fields (2.0 mm2). G1: grade 1; G2: grade 2; G3: grade 3; PanNEN: pancreatic neuroendocrine neoplasm; PanNEC: pancreatic neuroendocrine carcinoma; PanNET: pancreatic neuroendocrine tumor.
Well-differentiated panNET
  G1< 3< 2
  G23 - 202 - 20
  G3> 20> 20
Poorly-differentiated panNEC
  PanNEC G3> 20> 20
  Small cell type
  Large cell type
Mixed neuroendocrine-non-neuroendocrine neoplasm


Table 2. VIPoma Clinical Characteristics in Pediatric Patients Across Published Case Reports
VIP: vasoactive intestinal peptide; MRI: magnetic resonance imaging; CT: computed tomography.
Age (years old)/gender (M/F)7 months/M11/F12/M15/F2/F
SymptomDiarrhea, dehydrationSecretory diarrheaDehydration, diarrhea, dizziness and flushingDiarrhea, vomiting, fatigue and thirstDiarrhea, failure to thrive, weight loss
Fecal spending-2 L/day-8 - 10 L/day-
Serum potassium3.3 mEq/L2.9 mEq/L-1.7 mEq/L< 2 mEq/L
VIP serum166 pg/mL-134.5 pg/mL2,150 pg/mL2,030 pmol/L
Other markers--Gastrin (138 pg/mL)Normal-
Diagnostic methodExploratory laparotomyAbdominal MRIAbdominal MRIEndoscopic retrograde cholangiopancreatographyTriple-phase CT scan
Tumor location in pancreasTailBody - tailTailBody - tail-
Tumor size5.0 × 5.0 × 5.0 mm4 × 5 cm1.5 × 1.0 × 0.8 cm6 × 5 cm-
Metastasis location-Peripancreatic lymph nodesMesenteric lymph nodes, liverSpleen, lymph nodes-
ManagementPancreatectomyPancreatectomy + splenectomyDistal pancreatectomyDistal pancreatectomy (85%) + splenectomyExploratory laparotomy for tumor resection