A rare case with an intrapancreatic accessory splenic cyst

Introduction: Accessory (supernumerary) spleens are quite common, around 10% of the population in autopsy specimens.Their location varies: around the splenic hilum, in the omentum and the mesentery or within organs such as the adrenals and/or the pancreas. Intrapancreatic accessory spleens are quite frequent (17%), especially in the pancreatic tail. The presence though of an epidermoid cyst within an intrapancreatic accessory spleen is exceedingly rare. Method: A 63year-old woman presented with abdominal discomfort with no history of acute pancreatitis or abdominal trauma. Ultrasonography revealed a 12 cm cyst in the splenic hilum region and the patient was admitted for further evaluation. A cystic mass was detected at the tail of the pancreas by abdominal tomography scan. Blood counts and serum tumor markers were within normal range. Results: The patient was referred for surgical treatment and underwent distal pancreatectomy and splenectomy with a presumptive diagnosis of a solid pseudopapillary neoplasm (SPN) of the pancreas. Postoperative histopathological examination revealed an intrapancreatic epidermoid splenic cyst. The patient was discharged in good general condition. Conclusions: Epidermoid cysts in intrapancreatic accessory spleens are relatively rare, located almost exclusively at the tail of the pancreas and usually are asymptomatic. The role of serum tumor markers such as CEA and CA 19-9 for diagnosis is questionable. Since an epithelial splenic cyst in the pancreas lacks particular characteristics on radiological examination, it is especially difficult to differentiate it from a pancreatic cystic neoplasm. In conclusion, epidermoid cyst in an intrapancreatic accessory spleen should be considered in the differential diagnosis of pancreatic tail cystic tumors.