Adrenocortical carcinoma (ACC) is a rare neoplasm with very poor
prognosis despite the recent development of aggressive antitumor therapies. The cause
of adrenal cancer remains elusive, but some molecular mechanisms could be
responsible for its development. Target-specific therapies have been developed for a
number of human malignancies and have resulted in therapeutic benefits in some cancer
patients. However, these therapies are only effective in cases in which the
corresponding targets are expressed in tumor tissues. Molecular analysis has had a
significant impact on the understanding of the pathogenetic mechanism of ACC
development and the evaluation of prognostic and predictive markers, among which
alterations of the IGF system, the Wnt pathway, p53 and molecules involved in cancer
cell invasion properties and angiogenesis seem to be very promising. These molecular
markers may not just play a role in the biology of these tumors and have prognostic
implications, but can also be used as potential targets for treatment. The aim of this
chapter is to summarize the genetic and molecular events implied in the pathogenesis of
ACC and to highlight challenges to the development of anticancer agents in recent
patents.
Keywords: Adjuvant therapy, adrenocortical cancer, bevacizumab, biomarkers,
β-catenin, EGF, growth factors, IGF 1, markers, MEN 1, microRNA, mitotane,
molecular factors, mTOR, SF-1, sorafenib, sunitinib, targeted therapy, Wnt
pathway, VEGF.
