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Topic: Systemic
Therapy for Advanced
Cholangiocarcinoma: New
Options on the Horizon
Cholangiocarcinoma (CCA) is the second most common
primary hepatic malignancy after hepatocellular
Massimo Colombo, MD carcinoma, representing approximately 15% of all
primary cancers of the liver. With all the caveats of a
misclassification that may have occurred in the years
Chairman of EASL International Liver when iCCA was not coded separately from extra-hepatic
Foundation in Geneva, Switzerland. CCA, the incidence of CCA has been soaring globally,
in part paralleling the increasing burden of chronic
liver diseases. The tumor is notable for a number of
peculiarities which include a remarkable propensity to
evade early diagnosis and defy treatment, not to speak
of its well recognized epidemiological, anatomical and
biological heterogeneity. In consequence of its clinical
aggressiveness, limited treatment options and high
rates of recurrence after resection, this cancer displays
one of the highest mortality-to-incidence ratio of any
solid neoplasm. While the tumor is classified into three
anatomical subtypes, intra-hepatic (iCCA, 20% of all
CCAs), peri hilar and distal tumor, each of these subtypes
is associated to different risk factors, from cirrhosis to
flukes and bile duct stones, and has different clinical
trajectories, all in the context of a significant geographic
Stefano Cascinu, MD variability. The fact that the majority of CCAs have a
sporadic onset, has important clinical conse quences,
Chair, Unit of Oncology, IRCCS one above all the lack of screening causing detection of
San Raffaele Scientific most biliary tumors at an advanced stage. Notably, in
Institute, Milan, Italy. the frame of such a remarkable clinical heterogeneity,
the neoplastic biliary epithelium evolves a myriad of
genetic and epigenetic changes that have been thoroughly
investigated as potential markers for developing precision
medicine based treatments. Yet, none of these genetic
markers has proven to help early diagnosis, the only
pragmatic approach to override the dismal prognosis of
CCA. All in all, a better understanding of the concerned
risk factors and the identification of those molecular
stigmas that have pathogenic relevance, have had ground
breaking clinical consequences in the direction of a
precision medicine oriented management of patients with
CCA.
