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Pancreatic Vipoma As a Differential Diagnosis in Chronic Pediatric Diarrhea: a Case Report and Review of the Literature

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Pancreatic Vipoma As a Differential Diagnosis in Chronic Pediatric Diarrhea: a Case Report and Review of the Literature Case Report J Med Cases. 2021;12(5):195-201 Pancreatic VIPoma as a Differential Diagnosis in Chronic Pediatric Diarrhea: A Case Report and Review of the Literature Carolina Bonilla Gonzaleza, d, Juliana Rusinqueb, Camila Uribeb, Ailim Cariasc, Maria Luisa Contrerasa Abstract only 2.8 cases per a million inhabitants reported in patients under 30 years of age [1]. Moreover, the reports of accurate di- Chronic diarrhea is a common chief complaint in the pediatric popu- agnoses in the pediatric population are even more sporadic [2]. lation with a wide range of diagnostic differentials; as such, whilst NETs secrete vasoactive substances such as peptides and neu- suspecting common causes, less prevalent conditions tend to be rotransmitters in various tissues, including the lungs, bronchial overlooked, such as neuroendocrine tumor pathologies. VIPomas tree, thymus, gonads, thyroid and/or gastrointestinal tract [3]. are characterized by hypersecretion of the vasoactive intestinal pep- These tumors are classified as functional and nonfunctional. tide (VIP), causing watery diarrhea, hypokalemia, and achlorhydria. The former, depending on their location, induce hypersecretion Nonetheless, its low incidence rate in children makes it an easily un- of specific hormones leading to characteristic symptoms [3]. noticed pathology. Herein, we report a case of a 14-year-old female In patients under 20 years old, NETs of functioning pancreatic patient and a review of relevant literature. The patient complained localization (PNET) represent 30% of all NETs [4], with the of 7-month history of watery diarrhea, multiple emetic episodes, and most frequent causes representing the insulinoma, gastrinoma, relevant past medical history of multiple hospitalizations. Chronic glucagonoma, VIPoma and somatostatinoma [4, 5] (Fig. 1). diarrheal disease work-up studies, including a high VIP scintigram, Pancreatic VIPomas represent 3-8% of all pancreatic showed a lesion suggestive of a VIPoma-type neuroendocrine pancre- NETs, with an incidence of 0.2 - 0.5 case/1,000,000 inhabit- atic tumor. A distal pancreatectomy was performed with a complete ants [6]. As suggested by their names, these tumors are vasoac- resolution of the symptoms. When faced with a pediatric patient pre- tive intestinal peptide (VIP) secretors, and up to 30% of them senting with chronic secretory diarrhea and whose work-up studies simultaneously produce pancreatic polypeptide, calcitonin, rule out the most common pathologies, the possible presence of a gastrin, neurotensin, gastric inhibitory peptide, serotonin, so- neuroendocrine tumor as VIPoma should be considered. matostatin, and a growth hormone-releasing hormone (Fig. 2). The VIPoma can present itself as an isolated neoplasm or, ex- Keywords: Chronic diarrhea; Vasoactive intestinal peptide; Pancre- ceptionally, be associated with multiple endocrine neoplasia atic tumor; Pediatric types 1 (MEN1) [6]. Generally, the VIPoma is described as watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome (or “pan- creatic cholera”), characterized by watery diarrhea (> 7 L/ Introduction day), hypokalemia and achlorhydria. Pancreatic tumors are usually reported as a unique and symptomatic mass, greater than 3 cm in size, located in the tail of the pancreas in 75% The incidence of neuroendocrine tumors (NETs) is low, with of cases and with high incidence of metastasis at the time of diagnosis (up to 60-80%), particularly to lymph nodes, liver, kidneys or bone [7]. Manuscript submitted November 8, 2020, accepted November 26, 2020 Published online March 5, 2021 Case Report aPediatric Critical Care, University Hospital Fundacion Santa Fe de Bogota, Bogota, Columbia bUniversidad de los Andes, Bogota, Colombia A 14-year-old female patient with no prior medical history cPediatric Gastroenterology, University Hospital Fundacion Santa Fe de Bo- presented to the hospital complaining of 7 months of watery gota, Bogota, Columbia diarrhea, approximately 7 stools/day, associated with poorly dCorresponding Author: Carolina Bonilla Gonzalez, University Hospital Fun- dacion Santa Fe de Bogota, Av. 9 No 116-20 Consultorio 617, Bogota 110111, characterized abdominal pain, vomiting, hypoxia, arthralgia, Colombia. Email: [email protected] myalgia and unintentional weight loss of 11 kg. Initial work- up studies revealed hypokalemia (1.6 mEq/L) associated with doi: https://doi.org/10.14740/jmc3535 electrocardiographic changes, requiring initial intravenous po- Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org This article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits 195 unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited VIPoma as a Diagnosis in Pediatric Diarrhea J Med Cases. 2021;12(5):195-201 Figure 1. Clinical classification of neuroendocrine tumors (NETs). tassium chloride administration. Stool cultures and serologic antibodies transglutaminase 23 U/mL, and IgA gliadin 3.42 U/ testing for common bowel diseases were negative, and im- mL. Similarly, bone scan, endoscopy and colonoscopy all re- mune profile testing was completely normal: immunoglobulin vealed normal results. A (IgA) 1.23 U/mL, negative antinuclear antibodies (ANAs), Endocrinological profile was evaluated, with the follow- negative antineutrophil cytoplasmic antibodies (ANCAs), ing results: free T4 0.76 ng/dL, cortisol am 13.71 µg/dL, pm gliadin antibodies immunoglobulin G (IgG) 2.67 U/mL, IgG 5.02 µg/dL, adrenocorticotropic hormone (ACTH) 10.8 pg/ Figure 2. Pancreatic VIPoma. 196 Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org Bonilla Gonzalez et al J Med Cases. 2021;12(5):195-201 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). mL, aldosterone 65 pg/mL, and thyroid stimulating hormone ously in the proximal body of the pancreas. The resected pan- (TSH) 1.73 µIU/mL (all in normal ranges). Additionally, the creatic tissue was 9.5 × 5 × 2.9 cm, of which 0.5 × 0.3 cm was patient had normal gastrin levels (17.9 pg/mL), with a low immunohistochemically well differentiated (which primarily parathyroid hormone at 4.5 pg/mL, and serum copper 148 µg/ involved the proximal body and tail of the pancreas). Meta- dL (normal), zinc 84 µg/dL (normal), alpha 1 antitrypsin in static involvement was present in four of the nine resected stool 2 mg/g (normal), with an important VIP elevation: value peri-pancreatic lymph nodes. of 91.2 pmol/L. Microscopically, the tumor was characterized by cellular Due to the identified VIP elevation, a scintigraphy was proliferation with slight nuclear pleomorphism and scant cyto- performed, demonstrating a pancreatic lesion with prominent plasm, arranged in small trabeculae and pseudoacinar forma- octreotide uptake. The patient was hospitalized in the pedi- tions with perineural invasion, without endangering the sec- atric intensive care unit (PICU) due to persisting high fecal tion edge. Mitotic rate was below 2 mitoses/mm2. expenditure of about 7 to 10 L/day and severe hypokalemia, Immunohistochemistry showed a reaction for synapto- with important dehydration and electrolyte inbalance. Mag- physin, chromogranin and cytokeratin 19. Expression was netic resonance imaging (MRI) scans were performed, which not observed for insulin, glucagon, somatostatin, serotonin, identified two high uptake focal lesions adjacent and above gastrin or companion diagnostics. The reported Ki-67 index the posterior aspect of the pancreas body, measuring 20 ×16 was 6.9%. mm and 10 × 9 mm, with enhancement similar to that of pan- Using the World Health Organization (WHO) Stage III creatic tissue, which correlates with focal uptake scintigraphy and the American Joint Committee on Cancer (AJCC) metas- of octreotide compatible with a clinical diagnosis of VIPoma tasis classification systems, a final diagnosis of a functional (Fig. 3). pancreatic primary NET VIPoma Grade 2 was made (based The biliopancreatic endoscopic ultrasound reported a nor- on the tumor’s clinical, laboratory, radiologic, and histopatho- mal pancreatic head echo-structure without observable injury. logic (immunohistochemical) profile). Further classification of In the pancreatic body, four hypoechoic lesions were observed. VIPomas is detailed in Table 1, using the latest WHO 2017 These were described as homogeneous, round, well defined, classification system, in which final grade is determined based with the largest measuring 13 mm and the smallest 7 mm in on whichever index (Ki-67 or mitotic) places the tumor in the diameter. All reported lesions were compatible with NETs. A highest-grade category [8]. After surgery, the patient presented fine-needle aspiration biopsy was performed by transgastric symptomatic improvement, with complete resolution of diar- route, obtaining samples of the largest lesion. Immunohisto- rhea, and correction of electrolyte/acid-base profiles. A post- chemistry studies confirmed positivity for CAM5.2, synapto- operative MRI showed a residual lesion in the sella turcica, physin, and chromogranin, confirming the diagnosis. consisting of an incidental focal lesion in the pituitary gland, Initial management with octreotide resulted in dramatic later classified as a small self-limited
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