Malignant Transformation Arising Within Unusual and Rare Hepatic Lesions: Fibropolycystic Disease Form of Ductal Plate Malformation and Biliary Adenofibroma

Abstract

Cholangiocarcinoma is the second most common hepatobiliary cancer following hepatocellular carcinoma, and 20% to 25% are intrahepatic. We describe 2 cases of intrahepatic cholangiocarcinoma arising within unusual and rare hepatic lesions, fibropolycystic liver disease form of ductal plate malformation and biliary adenofibroma, whose association with malignancy is rarely reported in the literature.

Imaging features of biliary adenofibroma of the liver with malignant transformation: a case report with literature review

“…On unenhanced images, the imaging findings of most BAF with malignant features resembled those of benign BAF. Yet, it was found that several features showed in some malignant BAF cases, including multiple lesions, unilocular solid-cystic mass, restricted diffusion on DWI, obscure margin, peripheral edematous halo and pseudocapsule formation, have not been described in benign BAF [ 6 , 10 , 12 , 15 ]. As for the enhanced characteristics of BAF, 10 cases offered detailed information of imaging features on contrast-enhanced CT or MR images, including 2 cases of benign BAF and 8 cases of malignant BAF.…”

“…As for the enhanced characteristics of BAF, 10 cases offered detailed information of imaging features on contrast-enhanced CT or MR images, including 2 cases of benign BAF and 8 cases of malignant BAF. 2 patients [ 17 ] with benign BAF (100%) showed delayed enhancements, and 6 patients [ 6 , 9 , 11 , 13 , 15 ] with malignant BAF (75%) showed marked enhancements in the arterial phase and washout in the venous phase. Thus, we hypothesize that wash-in the arterial phase followed by wash-out in the venous phase is a typical imaging feature of malignant BAF.…”

Pathology and molecular pathology of cholangiocarcinoma

“…The "small duct type" iCCA can arise in the context of a ductal plate malformation or a biliary adenofibroma, as occasionally reported in the literature [4,5] . Biliary adenofibroma is a recently introduced category in the WHO 2019 [1] , representing a mass forming lesion made of glands and stroma, with cystic dilatation, covered by biliary cuboidal epithelium without significant atypia not communicating with the biliary tree [6] , and in fact after extensive sampling of the tumour it is possible to appreciate aspects resembling an underlying biliary adenofibroma [Figure 3A and B].…”

Magnetic Resonance Imaging Findings of Biliary Adenofibroma

“…thus far in the medical literature. 1,[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Previously reported cases focused on the clinical and pathological features of biliary adenofibroma, while the imaging findings were recorded only sporadically. Currently, MRI is the imaging modality of choice for evaluating a range of intrahepatic tumors.…”

“…Although biliary adenofibroma is classified as a benign biliary tumor and precursor in the 2019 WHO tumor classification system, 29 histopathological and biological spectra of biliary adenofibroma are unclear owing to its rarity. To date, biliary adenofibromas with benign behavior, 1,[6][7][8]13 carcinoma in situ, 10 and malignant transformation 4,5,12,[14][15][16]18 have been reported.…”