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Acinar cell cystadenoma, also known as an acinar cystic transformation of the pancreas, is an exceedingly rare but benign pancreatic lesion. A 51-year-old woman was transferred to Inje University Busan Paik Hospital because of an 8 cm-sized calcified, multiseptated, and multilocular cystic mass in the pancreatic tail observed during abdominal CT performed at another hospital. The patient did not complain of abdominal pain or other symptoms, and her laboratory findings were normal. MRI showed that the cyst was not connected to the main pancreatic duct. A pancreatic serous cystadenoma was suspected, and a laparoscopic distal pancreatectomy was performed. The resected mass was composed of variable sized multilocular cysts with incomplete septa and focally lined by epithelium with acinar differentiation. The patient was diagnosed with acinar cell cystadenoma and is currently being followed up regularly. No complications or recurrences have been observed.

Cystic lesions of the pancreas are a rare entity, and few reports have described their natural history in children. A previously published report described a 9-year-old boy with an acinar cell cystadenoma, discovered during a laparoscopic appendectomy. Initially asymptomatic and followed by serial MRI, this patient presented to our institution several years later with chronic obstructive symptoms that required surgical intervention. Planning for resection included multidisciplinary input from the genetics and endocrinology services. Initially, a partial pancreatic resection was performed in an attempt to preserve normal pancreatic tissue and islet cell function. Progression of the cystic disease and recurrent obstruction necessitated further surgical intervention. Here we present the updated clinical course and outcome of a 12-year-old boy who underwent a total pancreaticoduodenectomy for a symptomatic acinar cell cystadenoma.

In this report, we describe a case of hitherto unreported primary retroperitoneal acinar cell cystadenoma that morphologically and immunophenotypically resembled pancreatic acinar cell cystadenoma. Pancreatic acinar cell cystadenoma is a very uncommon benign lesion characterized by acinar cell differentiation, the evidence of pancreatic exocrine enzyme production, and the absence of cellular atypia. Our case occurred in a 55-year-old woman presenting a 10-cm multilocular cystic lesion in the retroperitoneum thought to be a mucinous cystic neoplasm. At laparotomy, the cystic mass, which showed no connection with any organ, was completely resected with a clinical diagnosis of cystic lymphangioma. The diagnosis of retroperitoneal acinar cell cystadenoma was based on the recognition of morphological acinar differentiation, the immunohistochemical demonstration of the acinar marker trypsin, and the absence of cellular atypia. These peculiar features can be used in the differential diagnosis with all the other cystic lesions of the retroperitoneum.