Adjuvant Therapies and Patient and Tumor Characteristics Associated With Survival of Adult Patients With Adrenocortical Carcinoma
Context:Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center.Objective:To identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies.Design:Patient data was collected in a retrospective single center study. Epidemiological, patient and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox-regression models (multivariable and univariable).Results:391 adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production (HR=1.4, HR=1.5), tumor stage (HR stage 3=2.1 and 2.1, HR stage 4=4.8) and tumor grade (HR=2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy, but did not impact overall survival. Patients with open adrenalectomy had improved overall survival.Conclusions:This study increases the evidence for adverse risk factors (cortisol production, high tumor stage and high tumor grade), and suggests the following therapy approach: Adrenocortical carcinoma patients should be treated with open adrenalectomy. Adjuvant therapy particularly mitotane therapy in conjunction with radiation should be considered in order to delay tumor recurrence.
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