Abstract
Abstract Introduction Adrenocortical carcinoma (ACC) is a rare neoplasm with a reported incidence of 0.5–2 cases per million population per year. Literature is scarce for this malignancy with poor prognosis, and treatment options have remained relatively limited. The objective of this report was to characterize patients and assess survival among patients with ACC managed at our institution. Methods A retrospective chart review (1996-2019) was performed on patients diagnosed with ACC confirmed via surgical pathology. Categorical variables were described using frequencies and percentages and continuous variables were described using means and standard deviations. Data was subsequently split into two time-periods defined by date of initial presentation. The first period (Time A) included 35 patients (1996-2007), and the second period (Time B) consisted of 17 patients (2008-2019). To compare time periods on continuous measures, two-sample t-tests were performed. Pearson chi-square tests and Fisher exact tests were used. Analysis was performed using SAS software (version 9.4; Cary, NC). Results A total of 52 patients were identified, 29 (55.8%) were female, and the mean age at surgery was 53 years. Overall, the most common presentations (25%, N = 13) were incidentaloma, abdominal pain and hormonal hypersecretion for each category. There was no statistically significant difference in the reason for presentation between the two time periods. Laboratory evidence of hormonal hypersecretion was identified in 21 (40.4%) patients, but biochemical profiles were not available in 8 (15.3%). Among patients found to have evidence of hormonal hypersecretion, elevated 24-hour urinary fractioned cortisol was the most common finding in both Time A and B [N=8 (22.9%)] and [N=7 (41.2%)], respectively, p = 0.2. With respect to post-operative management, 20 (38.5%) received mitotane, 13 (25%) received other chemotherapy, while 3 (5.8%) received external radiation of tumor bed, with no statistical difference between the two time periods in these parameters. Among those with surgery more than 5 years ago, the overall 5-year survival rate was 53.1% (26/49), and 40% (14/35) and 85.7% (12/14) for Time A and B respectively (p = 0.004) Conclusion Overall, the most common presentation of ACC in our report was found to be equally distributed amongst incidentaloma, hormonal hypersecretion, and abdominal pain. In hormonal hypersecretion, hypercortisolism, a recognized poor prognosticator, was the most frequent. The 5-year survival rate for ACC was noted to be poor but the reason for the significant improvement in the survival rates in Time B vs Time A is unclear and requires further investigation. The most common post-operative adjuvant therapy was mitotane. Further research is necessary for earlier detection, and for developing different management strategies in order to improve outcomes in patients with ACC. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.
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