Curious case of paediatric multiple pancreatic cysts

Abstract

DescriptionIsolated pancreatic cysts are extremely rare in children and often pose certain diagnostic challenges. This is especially true when they are large in size and cause mass effect on adjacent organs. In these cases, the main differential diagnostic considerations apart from primary pancreatic cystic lesions would include duplication cysts or retroperitoneal cystic lymphangiomas, with the latter differentials more common in the paediatric population. We present a case of a child with multiple pancreatic cysts, with radiological appearance mimicking cystic lymphangioma in the upper abdomen. Case ReportTwenty-month-old child presented with intermittent abdominal discomfort and a palpable mass on the left side of the abdomen.The child is otherwise well. She was born from nonconsanguineous parents with no known family history of genetic disorders. Ultrasound demonstrates multilocular cystic structure at the left flank with internal septa (Fig. 1). There was no solid component or internal vascularity seen. It abuts the left kidney, but does not seem to arise from it. The sonographic appearance favours a non-aggressive lesion, with cystic lymphangioma being the main consideration.Subsequently computed tomography abdomen followed by magnetic resonance imaging abdomen (Fig. 2) were performed, which confirm the presence of a large, multiseptated cystic lesion involving the pancreatic body and tail. There is mass effect with indentation and displacement of the gastric body and the left kidney. The pancreatic body/tail appears to be replaced by the lesion. Although atypical, the main consideration at reporting was that of a cystic lymphangioma.Decision was made to proceed to diagnostic aspiration and injection of sodium taurocholate 3% into the cyst. Clear fluid was aspiration and contrast was subsequently injected into the locules, showing some communications with each other. The aspirated fluid shows extremely high amylase (45 341 U/L) and lipase level (335 200 U/L), suggestive of cysts of pancreatic origin with possible communication with the pancreatic duct.Patient then underwent spleen preserving distal pancreatectomy. The cut sections show absence of normal pancreatic parenchyma which is replaced by multiple thin walled cysts ranging from 0.3 to 5.5 cm containing haemoserous fluid (Fig. 3).Microscopically, these cysts are lined by a single layer of bland cuboidal to flattened ductal-type epithelium with surrounding fibrosis. There is intervening scant atrophic pancreatic parenchyma. No atypia, necrosis or mitotic activity seen. No evidence of malignancy.Patient recovered well post-operatively and was discharged a few days afterwards. Concerns were raised whether patient has underlying syndromes, in particular von Hippel-Lindau (VHL). Patient's family, however, declined genetic testing.