Molecular and genetic characteristics of adrenocortical cancer
Byakhova M.M.¹, Voronkova I.A.^1,2, Krivosheev A.B.<sup>2

1</sup> Moscow Regional Research and Clinical Institute named after M.F. Vladimirskiy, Moscow
² Endocrinological Scientific Center, Moscow

Adrenocortical cancer is a rare cancer originating from adrenal cortex which is characterized by aggressive clinical course, late diagnosis, and poor clinical outcomes. The occurrence of adrenocortical cancer is 0.5 to 2 cases per million per year. Molecular genetics of this condition remains elusive. Adrenocortical cancer is diagnosed both as a sporadic disease and as a component of some inherited syndromes. The penetrance of this disorder is low thus significantly reducing the possibility to follow hereditary nature of adrenocortical cancer. For many decades, molecular studies involved little samples and investigated genes which promote the development of this disorder. It was demonstrated that elevated expression of IGF2, inactivating mutation of TP53, and activation of Wnt/b-catenin signal pathway due to the mutation of b-catenin gene are associated with adrenocortical cancer. This paper summarizes data on key molecular genetic mechanisms of adrenocortical cancer.

Key words: adrenocortical tumors, adrenocortical cancer, molecular pathology, genetic pathology, signal pathway, inherited syndromes.
For citation: Byakhova M.M., Voronkova I.A., Krivosheev A.B. Molecular and genetic characteristics of adrenocortical cancer // RMJ. 2017. № 22. P. 1651–1653.