Presentation
History of bilateral lung transplant for cystic fibrosis on immunosuppressive medication. 18 month history of drenching sweats. No other symptoms or examination findings.
Patient Data
CT shows sternal wiring from bilateral lung transplant.
Fatty replacement noted in the pancreas consistent with a history of cystic fibrosis.
Large cystic lesion occupying most of the left lobe of the liver which has internal enhancing septations and apparent communication to the biliary tree with communicating dilated intrahepatic ducts and a dilated common duct.
MRCP confirms the findings on CT, particularly the biliary communication. The sphincter of Oddi appears open suggestive of mucin hypersecretion.
No solid nodules or "thread sign" demonstrated on MRI imaging.
Case Discussion
Intraductal papillary neoplasm of the bile duct (IPNB) was suspected on imaging and the patient underwent surgical resection which confirmed the diagnosis histologically. Biliary communication and lack of ovarian-like stroma (OLS) on histology were key to diagnosis.
Unable to process the form. Check for errors and try again.