Clinical Development in Challenging Cancers: Adrenocortical Carcinoma

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In this first article of four, our Medics take a closer look at Adrenocortical Carcinoma, treatment of this rare type of cancer and future prospects based on Immunotherapy.

“Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 1 to 2 patients per million people annually [1-3], and one in which most patients, at diagnosis, already present with a locally advanced or metastatic disease not suitable for surgery [4]. Age distribution for this disease follows a bimodal pattern, with an initial peak in childhood, and a subsequent peak in adults between the fourth and fifth decades [5] [Fig. 1]. Higher incidence rates (3-4 cases per million annually) have been described in Southern Brazil, particularly in children below the age of 15 years [6]. While genetic aberrations may predispose to adrenal cortical tumor formation, it has been suggested that environmental factors, such as pesticides used in agricultural activities, also play a pivotal role in tumorigenesis in Southern Brazil [6], given that the distribution of the disease follows a regional rather than familial pattern [7].”

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