Adrenocortical Carcinoma: Clinical Presentation, Outcomes of Treatment and Survival Analysis from a 15-Year Experience
Nath Chawaltonpipat¹, Apirak Santi-ngamkun¹, Julin Opanuraks¹, Kamol Panumatrassamee¹
Affiliation : ¹ Division of Urology, Department of Surgery, Faculty of Medicine, Chulalongkorn University, King Chulalongkorn Memorial Hospital, Bangkok, Thailand
Background: Adrenocortical carcinoma (ACC) is a rare malignant tumor of adrenocortical origin, often with an unfavorable survival. The estimated incidence is 0.7 to 2 per million per year.
Materials and Methods: The authors retrospectively reviewed all clinical data of the ACC patients that underwent surgical extirpation in the present study institution between January 2005 and December 2020. All demographic data, perioperative outcomes, survival, and predictive factors associated with mortality were analyzed.
Results: Twenty-three patients were included in the present study. The median (IQR) age was 51 (38 to 61) with females predominant at 78.3%. The median (IQR) tumor size was 8.3 (5.3 to 14.8) cm. with the left side predominant at 65.2%. The most common presentation was hormone-related symptoms at 52.2% and isolated hypercortisolism at 34.8%, was the most common hormonal excess. Complete resection (R0) was achieved in 82.6% of the patients. Following the European Network for the Study of Adrenal Tumors (ENSAT) staging system, 52.2% of the patients were in stage 2. Adjuvant therapies such as radiation, mitotane, and chemotherapy, were required in 47.7% of the patients. Eight patients (34.8%) were death during the median follow up of 44 months. The estimated 1-, 2-, and 5-year overall survivals were 78.3%, 73.9%, and 65.2% respectively. The ENSAT stage 3 to 4 (HR 1.61, 95% CI 1.16 to 21.5, p=0.03), nodal metastasis (HR 2.11; 95% CI 1.36 to 50.26; p=0.02), distant metastasis (HR 1.75; 95% CI 1.16 to 28.62; p=0.03), and the neutrophil to lymphocyte ratio (NLR) of 5 or more (HR 2.66; 95% CI 1.74 to 116.81; p=0.01) were significant factors associated with disease mortality in univariate analysis. NLR of 5 or more was the only significant factor (HR 2.51, 95% CI 1.2 to 126.84; p=0.03) in multivariate analysis.
Conclusion: ACC is a rare malignancy associated with aggressive behavior. Multidisciplinary approach with surgical removal is the mainstay of treatment. The high NLR of 5 or more is a significant factor associated with poor disease survival.
Received 27 June 2022 | Revised 30 August 2022 | Accepted 5 September 2022
DOI: 10.35755/jmedassocthai.2022.11.13704
Keywords : Adrenocortical carcinoma; Mitotane; Survival; Prognosis; Neutrophil to lymphocyte ratio
