Adrenocortical carcinoma (ACC) is a rare disease with an age-adjusted incidence of 1.02 patients per million in the United States and an aggressive phenotype with poor outcomes. Surgery can be curative, but this is not always possible given advanced stage at presentation (70% present with Stage III and Stage IV disease) and patients often require mitotane along with cytotoxic chemotherapy.
Despite increased knowledge of its heterogeneous molecular background, translating that insight to systemic therapies has not been successful. Due to its rarity, conducting clinical trials have also been challenging and the success thus far has been based upon multicenter and international collaboration. Much of what we know now is based upon retrospective reviews.
This topic aims to bring together multiple voices around the evaluation and treatment of ACC, including novel therapies, current standard of care, and future directions.
The scope of this includes surgical approaches to adrenalectomy surgery, ablative technologies for metastatic disease, surgical treatment for metastatic disease, radiotherapy as adjuvant treatment for adrenal cancer, systemic therapy, mitotane use, treatment of complications from the treatments, treatment of the hormonal producing tumors, biochemical evaluation. In addition, we will include the molecular evaluation, models such as organoids for studying these tumors, as well as what we have learned from translational models in ACC. The future direction of care of these patients is of great interest as well.
Adrenocortical carcinoma (ACC) is a rare disease with an age-adjusted incidence of 1.02 patients per million in the United States and an aggressive phenotype with poor outcomes. Surgery can be curative, but this is not always possible given advanced stage at presentation (70% present with Stage III and Stage IV disease) and patients often require mitotane along with cytotoxic chemotherapy.
Despite increased knowledge of its heterogeneous molecular background, translating that insight to systemic therapies has not been successful. Due to its rarity, conducting clinical trials have also been challenging and the success thus far has been based upon multicenter and international collaboration. Much of what we know now is based upon retrospective reviews.
This topic aims to bring together multiple voices around the evaluation and treatment of ACC, including novel therapies, current standard of care, and future directions.
The scope of this includes surgical approaches to adrenalectomy surgery, ablative technologies for metastatic disease, surgical treatment for metastatic disease, radiotherapy as adjuvant treatment for adrenal cancer, systemic therapy, mitotane use, treatment of complications from the treatments, treatment of the hormonal producing tumors, biochemical evaluation. In addition, we will include the molecular evaluation, models such as organoids for studying these tumors, as well as what we have learned from translational models in ACC. The future direction of care of these patients is of great interest as well.
