TY - JOUR
T1 - Malignant transformation of biliary adenofibroma
T2 - A rare biliary cystic tumor
AU - Thompson, Scott M.
AU - Zendejas-Mummert, Benjamin
AU - Hartgers, Mindy L.
AU - Venkatesh, Sudhakar K.
AU - Smyrk, Thomas C.
AU - Mahipal, Amit
AU - Smoot, Rory L.
N1 - Publisher Copyright:
© Journal of Gastrointestinal Oncology.
PY - 2016
Y1 - 2016
N2 - Biliary adenofibromas (BAFs) are rare, benign biliary cystic tumors with potential for malignant transformation. Of the eleven prior cases of BAF reported in the literature, six showed evidence of malignant transformation. We describe the clinical, imaging and pathology features of two cases of malignant BAF and review the existing literature to raise awareness of this entity and provide additional tools for diagnosing this rare tumor Additionally, we identified a loss of function mutation in the cyclin-dependent kinase inhibitor 2A (CDKN2A) tumor suppressor gene in a malignant caudate lobe BAF, thereby providing potential insight into the molecular pathogenesis of BAF malignant transformation. Although additional cases and longer-term follow-up are needed, our cases suggest that recurrence or metastasis of malignant BAF is not common and that complete surgical resection can be curative.
AB - Biliary adenofibromas (BAFs) are rare, benign biliary cystic tumors with potential for malignant transformation. Of the eleven prior cases of BAF reported in the literature, six showed evidence of malignant transformation. We describe the clinical, imaging and pathology features of two cases of malignant BAF and review the existing literature to raise awareness of this entity and provide additional tools for diagnosing this rare tumor Additionally, we identified a loss of function mutation in the cyclin-dependent kinase inhibitor 2A (CDKN2A) tumor suppressor gene in a malignant caudate lobe BAF, thereby providing potential insight into the molecular pathogenesis of BAF malignant transformation. Although additional cases and longer-term follow-up are needed, our cases suggest that recurrence or metastasis of malignant BAF is not common and that complete surgical resection can be curative.
KW - Biliary adenofibroma (BAF)
KW - Caudate lobe
KW - Malignant transformation
KW - Next generation sequencing
KW - Tumor suppressor
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U2 - 10.21037/jgo.2016.09.14
DO - 10.21037/jgo.2016.09.14
M3 - Article
AN - SCOPUS:85007009240
SN - 2078-6891
VL - 7
SP - E107-E112
JO - Journal of Gastrointestinal Oncology
JF - Journal of Gastrointestinal Oncology
IS - 6
ER -