Publication Date

10-2022

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Abstract

Introduction: Intraductal Papillary Mucinous Neoplasms (IPMNs) of the pancreas are premalignant, intraductal, mucin secreting cystic lesions. IPMNs are a rare entity in the pediatric population and commonly seen in men in their 6 to 7 decade. Pediatric IPMN has been previously reported in a 14-year-old and a neonate, who also had hyperinsulinism and a de novo germline SKIL gene mutation. . Kim et al. did a ten-year single centered pediatric pancreatic malignancy study discovering IPMN in one patient of 35 with pancreatic mass removals . Case: Our patient is an 18-year-old male who initially presented at the age of 15 with acute, then recurrent mild pancreatitis. His routine screening investigations including calcium, triglycerides, and IgG4 levels were normal. No mutations were detected upon pancreatitis gene panel including PRSS1, SPINK1, CFTR, CASR, CTRC, and CPA1 genes. The gallbladder was normal on abdominal ultrasonography, however Magnetic Resonance Cholangio Pancreatography (MRCP) showed focal irregular dilation of the pancreatic duct with a concern of santorinocele, which is a cystic dilation of the dorsal pancreatic duct.[ATM1] The patient underwent Endoscopic Retrograde Cholangio-Pancreatography (ERCP), and Endoscopic Ultrasound (EUS) with findings of mucin seen in the major papilla with a “fisheye” appearance (fig 1) and a cystic lesion measuring 7x22mm communicating with the pancreatic duct in the head of the pancreas (fig 2), respectively. Cytology was obtained through EUS guided fine-needle aspiration as well as aspirating mucus from the pancreatic duct during ERCP and both samples confirmed IPMN[ATM2] . Discussion: IPMN was first described in the 1980s but is increasingly recognized with the current incidence estimated as 0.48-2.04 per 100,000 . Pediatric IPMN is a premalignant lesion and typically progresses from IPMN adenoma, borderline dysplastic, carcinoma-in-situ and eventually to invasive carcinoma, usually within 5 years. IPMNs lesions are classified as main duct (MD-IPMN), branch duct (BD-IPMN) or mixed type with MTIPMN harboring a higher risk of malignant transformation. IPMNs are often diagnosed incidentally on imaging studies but can also present with abdominal pain or recurrent pancreatitis. It has been reported that patients presenting with pancreatitis are at high risk of harboring a malignancy . Worrisome Clinicopathologic features include cyst size ≥ 3 cm, thickened or enhancing cyst walls, main duct size 5–9 mm, non-enhancing mural nodules, an abrupt change in pancreatic duct caliber with distal pancreatic atrophy and lymphadenopathy. In addition, the incidence of extra-pancreatic neoplasms is higher in patients with IPMN, with reported rates of 25% to 50% Conclusion: Despite the rarity and low incidence of pediatric pancreatic tumors, especially IPMNs, the broad differential diagnosis of pediatric pancreatic lesions associated with pancreatitis has to include IPMNs especially in view of the potential malignant transformation.

Disciplines

Gastroenterology | Pediatrics

Notes

Presented at the North American Society for Pediatric Gastroenterology, Hepatology & Nutrition (NASPGHAN) Annual Conference; October 12-15, 2022; Orlando, Florida.

Intraductal Papillary Mucinous Neoplasm In An 18-year-old With Recurrent Pancreatitis

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