Advanced Adrenocortical Carcinoma: From Symptoms Control to Palliative Care

Simple Summary Adrenocortical cancer (ACC) is a rare malignancy, often diagnosed late and with a poor prognosis. Currently, ACC best management is achieved in referral centers, where a multidisciplinary approach (endocrinologists, oncologists, surgeons, radiologists and radiotherapists) can intercept the course of a patient with ACC early and operate with life-prolonging intents. Even in cases of advanced disease, multimodal treatments (chemotherapy and mitotane, surgery and/or radiotherapy) and skillful management of the medical complications of ACC can ensure significant improvements in survival. However, patients with advanced ACC suffer from relevant psychophysical symptoms and experience significant losses in quality of life. There is now robust evidence that the early integration of supportive and palliative care in standard oncological management may relieve cancer patients' burden, mediate aggressive treatments and improve quality of life, and not only in the end-of-life period. In this paper, we provide an up-to-date literature review on the role of supportive and palliative care in ACC management. The prognosis of patients with advanced adrenocortical carcinoma (ACC) is often poor: in the case of metastatic disease, five-year survival is reduced. Advanced disease is not a non-curable disease and, in referral centers, the multidisciplinary approach is the standard of care: if a shared decision regarding several treatments is available, including the correct timing for the performance of each one, overall survival is increased. However, many patients with advanced ACC experience severe psychological and physical symptoms secondary to the disease and the cancer treatments. These symptoms, combined with existential issues, debase the quality of the remaining life. Recent strong evidence from cancer research supports the early integration of palliative care principles and skills into the advanced cancer patient's trajectory, even when asymptomatic. A patient with ACC risks quickly suffering from symptoms/effects alongside the disease; therefore, early palliative care, in some cases concurrent with oncological treatment (simultaneous care), is suggested. The aims of this paper are to review current, advanced ACC approaches, highlight appropriate forms of ACC symptom management and suggest when and how palliative care can be incorporated into the ACC standard of care.

Automated summary

Adrenocortical cancer (ACC) is a rare malignancy with a poor prognosis. The best management approach involves a multidisciplinary team and can improve survival. Even in advanced cases, multimodal treatments and skillful management can lead to significant improvements in survival. However, patients with advanced ACC often suffer from severe physical and psychological symptoms and experience a decline in quality of life. The early integration of supportive and palliative care can alleviate the burden on cancer patients, mediate aggressive treatments, and improve their quality of life.