Abstract
Insight into the health-related quality of life (HRQoL) impact of adrenocortical carcinoma (ACC) is important. The disease and its treatment options potentially have an impact on HRQoL. For patients with limited survival, HRQoL research is of utmost importance. We will therefore provide an overview of HRQoL studies in patients with ACC. We found six studies that measured HRQoL in 323 patients with ACC (3 cross-sectional, 1 cohort, 2 trials), all indicating a reduced HRQoL compared to the general population. The FIRMACT trial found that HRQoL of patients with ACC was reduced compared to the general population, and that chemotherapy-mitotane further reduced HRQoL even though survival improved. Clinical aspects of the disease, including cortisol and aldosterone production and adrenal insufficiency have shown great impact on HRQoL in benign disease, even after the recovery of hormonal status. However, the impact of malignant adrenal disease and treatment options on HRQoL including adrenalectomy, radiotherapy, mitotane therapy, and chemotherapy have not been sufficiently studied in patients with ACC. Although the number of HRQoL studies in patients with ACC is limited, the existing literature does indicate that ACC has a large impact on patients’ HRQoL, with disease specific aspects. Further HRQoL research in patients with ACC is essential to improve patient-centered care, preferably by using an ACC-specific HRQoL questionnaire.
Highlights
Adrenocortical carcinoma (ACC) is a rare cancer with an incidence between 0.7 and 2.0 per million persons per year [1]
ACC presents with autonomous adrenal hormone production in 50–60% of the cases, most commonly with cortisol production or a combination of cortisol and androgen production [18]
Patients suffer from other issues, such as changes in body image, health behavior, fatigue, negative health outlook, and financial insecurity. These effects on health-related quality of life (HRQoL) have been studied in other types of cancer, but we have found no studies that included patients with ACC
Summary
Adrenocortical carcinoma (ACC) is a rare cancer with an incidence between 0.7 and 2.0 per million persons per year [1]. The overall median survival ranges from 5 to 159 months, and is strongly dependent on the stage of disease. ACC can present as a functional or nonfunctional tumor, with the latter often being an additional finding on imaging done for other purposes (incidentaloma finding). Treatment for ACC depends on the stage of disease [2,3]. The optimal treatment for solitary tumors is surgical resection. In cases with a high risk of recurrence (stage III, R1 or Rx resection, Ki67 > 10%), surgery is followed by at least 2 years of adjuvant mitotane monotherapy. For metastatic disease or irresectable tumors, the treatment consists of mitotane monotherapy, radiotherapy, or chemotherapy with or without the addition of mitotane
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