A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature

Abstract Background Biliary adenofibroma is an exceptionally rare benign liver tumor with the potential for malignant transformation. In literature, only 21 cases have been described. Clinical presentation In a healthy 63-year-old woman, a partly solid, partly cystic mass in the left lobe of the liv...

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Main Authors: Anne-Kathrin Sturm, Thilo Welsch, Christoph Meissner, Daniela E. Aust, Gustavo Baretton
Format: Article
Language:English
Published: Japan Surgical Society 2019-06-01
Series:Surgical Case Reports
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Online Access:http://link.springer.com/article/10.1186/s40792-019-0661-2
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author Anne-Kathrin Sturm
Thilo Welsch
Christoph Meissner
Daniela E. Aust
Gustavo Baretton
author_facet Anne-Kathrin Sturm
Thilo Welsch
Christoph Meissner
Daniela E. Aust
Gustavo Baretton
author_sort Anne-Kathrin Sturm
collection DOAJ
description Abstract Background Biliary adenofibroma is an exceptionally rare benign liver tumor with the potential for malignant transformation. In literature, only 21 cases have been described. Clinical presentation In a healthy 63-year-old woman, a partly solid, partly cystic mass in the left lobe of the liver during a routine ultrasound examination was found. The computed tomography (CT) scan of the abdomen showed a 6.3 × 5.0-cm multilobulated cystic, partly hypervascularized mass in the liver segment IVa, with extension into segments II and IVb. There was no evidence of lymph node or distant metastases. Extirpation of the tumor was indicated by the multidisciplinary tumorboard. Microscopic examination showed a biphasic composed tumor with tubules embedded in fibrous stroma. In addition, there were also areas with pseudopapillary projections, as well as parts with focal cribriform-like growth pattern, which have been indicated as a possible sign of malignant transformation. Additionally, we found two different polymorphisms in the encoded TP53 und KIT in both distinct morphology tumor areas by molecular analysis, which ensured a tumor in malignant transformation. The patient has been alive for 24 months after R0 resection without tumor recurrence. Further investigation of more cases of this rare entity is necessary to proof molecular genesis. Conclusions We report a rare case of a biliary adenofibroma with transition to an intrahepatic cholangiocellular carcinoma and present a brief literature review.
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spelling doaj-art-c0f3929b59cd4d8ba496c2f52daa0b9c2025-08-02T18:31:03ZengJapan Surgical SocietySurgical Case Reports2198-77932019-06-01511610.1186/s40792-019-0661-2A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literatureAnne-Kathrin Sturm0Thilo Welsch1Christoph Meissner2Daniela E. Aust3Gustavo Baretton4Institute for Pathology, University Hospital Carl Gustav Carus, TU DresdenDepartment of Visceral, Thoracic and Vascular Surgery, University Hospital Carl Gustav Carus, TU DresdenDepartment of Radiology, University Hospital Carl Gustav Carus, TU DresdenInstitute for Pathology, University Hospital Carl Gustav Carus, TU DresdenInstitute for Pathology, University Hospital Carl Gustav Carus, TU DresdenAbstract Background Biliary adenofibroma is an exceptionally rare benign liver tumor with the potential for malignant transformation. In literature, only 21 cases have been described. Clinical presentation In a healthy 63-year-old woman, a partly solid, partly cystic mass in the left lobe of the liver during a routine ultrasound examination was found. The computed tomography (CT) scan of the abdomen showed a 6.3 × 5.0-cm multilobulated cystic, partly hypervascularized mass in the liver segment IVa, with extension into segments II and IVb. There was no evidence of lymph node or distant metastases. Extirpation of the tumor was indicated by the multidisciplinary tumorboard. Microscopic examination showed a biphasic composed tumor with tubules embedded in fibrous stroma. In addition, there were also areas with pseudopapillary projections, as well as parts with focal cribriform-like growth pattern, which have been indicated as a possible sign of malignant transformation. Additionally, we found two different polymorphisms in the encoded TP53 und KIT in both distinct morphology tumor areas by molecular analysis, which ensured a tumor in malignant transformation. The patient has been alive for 24 months after R0 resection without tumor recurrence. Further investigation of more cases of this rare entity is necessary to proof molecular genesis. Conclusions We report a rare case of a biliary adenofibroma with transition to an intrahepatic cholangiocellular carcinoma and present a brief literature review.http://link.springer.com/article/10.1186/s40792-019-0661-2Biliary adenofibromaBile duct adenomaIntrahepatic cholangiocarcinomaDuctal plate malformationLiver
spellingShingle Anne-Kathrin Sturm
Thilo Welsch
Christoph Meissner
Daniela E. Aust
Gustavo Baretton
A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
Surgical Case Reports
Biliary adenofibroma
Bile duct adenoma
Intrahepatic cholangiocarcinoma
Ductal plate malformation
Liver
title A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
title_full A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
title_fullStr A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
title_full_unstemmed A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
title_short A case of biliary adenofibroma of the liver with malignant transformation: a morphomolecular case report and review of the literature
title_sort case of biliary adenofibroma of the liver with malignant transformation a morphomolecular case report and review of the literature
topic Biliary adenofibroma
Bile duct adenoma
Intrahepatic cholangiocarcinoma
Ductal plate malformation
Liver
url http://link.springer.com/article/10.1186/s40792-019-0661-2
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