Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, with a poor prognosis and no significant new treatment options on the horizon. The best option for cure is surgery. Treatments for metastatic disease rely on the adrenolytic agent mitotane and combination chemotherapy, which are rarely successful.

Lalli & Luconi have reviewed state of the art knowledge of the molecular, immunohistochemical and genomic characterisation of ACC. They describe new immunohistochemistry markers such as the Ki67 labelling index, Wnt, GLUT1 and SF-1. New molecular markers include finding loss of heterozygosity of the imprinted 11p15 region with a deletion of the maternal allele and a consequent overexpression of IGF2, which may be related to overall survival.

They also describe the concept of a ‘liquid biopsy’: detecting circulating markers of malignancy such as circulating tumour cells, miRNAs, exosomes and circulating cell-free DNA of tumour origin which may provide an early sign of disease recurrence or metastases. Excitingly, a recent mouse model of ACC may provide a means of studying new therapeutic options.

This detailed characterisation of ACC, together with new animal models, will hopefully provide a platform for greater understanding of ACC biology, and development of more successful treatment options to give hope to our patients.

Read the full article in Endocrine-Related Cancer 25 R31–R48