A rare case of extrahepatic biliary mucinous cystic neoplasm in a middle-aged woman: A case report.

Biliary mucinous cystic neoplasms are very rare tumors of the biliary tract with malignant potential. Preoperative diagnosis is challenging, as clinical, biochemical and radiological features are not specific. Surgical resection with negative margins is the gold standard treatment for these uncommon lesions. A 55-year-old woman presented at the Third Department of Surgery (Attikon University Hospital, Athens, Greece) with a history of mild right upper quadrant (RUQ) abdominal pain and jaundice. A 2-cm lesion in the distal common bile was identified by imaging. Following discussion in our multidisciplinary board meeting the patient underwent a pylorus preserving pancreatoduodenectomy, and histopathological examination revealed an ovarian-stromal type intraductal mucinous cystic neoplasm of the extra hepatic biliary. Since biliary mucinous cystic neoplasms are characterized by malignant transformation and high rates of recurrence, surgical resection with negative margins is the treatment of choice for both non-invasive and invasive biliary mucinous cystic neoplasms.