Repeated Negative Biopsies in Isolated High-Grade Cystic Duct Dysplasia with Progression to Adenocarcinoma

Cystic and bile duct dysplasia is a rare histological finding, especially when found in the absence of an underlying malignancy. We report a patient who presented with jaundice and weight loss. Clinical and cytological evidence suggested a diagnosis of cholangiocarcinoma and the patient underwent a pancreatico-duodenectomy. Histopathological examination suggested a diagnosis of two foci of biliary dysplasia: cystic duct and lower common bile duct. Fifteen months later, the patient re-presented with signs of obstructive jaundice and biliary sepsis. Although CT scan revealed images highly indicative of metastatic disease, repeated biopsies failed to confirm this. Eventually a liver biopsy did reveal moderately differentiated adenocarcinoma, however oncological interventional was no longer appropriate and the patient was managed palliatively. This case report focuses on the current understanding of progression of biliary dysplasia.