Adrenal tumor size and the severity of cortisol excess are associated with a higher risk of metastatic disease at diagnosis, according to a study recently published in The Journal of Clinical Endocrinology & Metabolism.
Researchers led by Marta Arajuo-Castro, of Hospital Universitario Ramon y Cajal in Madrid, Spain, point out in their article, “Severity and management of hypercortisolism in patients with adrenocortical carcinoma and overt Cushing´s syndrome,” that Cushing’s syndrome (CS) due to adrenocortical carcinoma (ACC) is a rare but highly aggressive condition in which excess cortisol may worsen oncological outcomes.
Up to 70% of ACCs are hormonally active, according to the authors. “Several studies have shown that ACC with associated CS is characterized by adverse prognosis, higher recurrence rates, and worse survival than nonfunctioning ACCs,” the authors write.
“Future prospective studies are needed to better define the prognostic role of cortisol excess severity and to optimize therapeutic strategies for controlling hypercortisolism in this challenging patient population.”
But there are few data about biochemical outcomes in patients with CS due to ACC – most studies have been focused in survival, the authors note. “The present study aimed to describe real-world management strategies for hypercortisolism in patients with ACC and to provide an overview of the biochemical response achieved with the different medical therapies used in routine clinical practice and to evaluate the association between the degree of hypercortisolism and survival outcomes in patients with ACC-associated CS,” they write.
For this study, researchers included 101 patients with ACC in an international retrospective to evaluate the prognostic role of baseline hypercortisolism on survival outcomes, including progression-free survival (PFS), disease-free survival (DFS), and overall survival (OS). “This international study provides clinically relevant evidence on how the severity of hypercortisolism and its management influence survival,” the authors write. “We found that higher baseline cortisol excess was associated with an increased risk of recurrence or progression, supporting its role as a marker of tumor aggressiveness.”
The authors write that several key findings emerged from this study:
- Adrenalectomy was linked to a high rate of biochemical remission in localized disease and offered partial endocrine control even in cases of metastasis
- The severity of hypercortisolism was 18 associated with an increased risk of recurrence/progression
- Although overall survival did not differ by cortisol severity, older age, UFC, hypokalemia, R0 after surgery, and Ki-67 were independent predictors of recurrence/progression and may help refine prognostic stratification
“Together, these findings highlight the clinical importance of early detection and aggressive treatment of cortisol excess in ACC,” the authors write. “Future prospective studies are needed to better define the prognostic role of cortisol excess severity and to optimize therapeutic strategies for controlling hypercortisolism in this challenging patient population.”
