Introduction:

Adrenocortical carcinoma is a rare endocrine neoplasm, but with highly aggressive behavior and a poor prognosis. Given its low prevalence, the experience of reference centers is essential to characterize the factors associated with this disease.

Methods:

It is a case series of patients with adrenocortical carcinoma, treated at a reference oncology institute between January 2007 and December 2017. The clinical and histopathological characteristics of patients are described. Progression-free survival and overall survival (OS) were estimated graphically and with time-to-event data using the Kaplan-Meier function.

Results:

19 patients were identified; 14 of them were women with a mean age at diagnosis of 43.4 years (range 20-65). 58% of the patients had hormone secretion, with Cushing's syndrome being the predominant one. 7 patients had metastatic compromise at the time of diagnosis. All patients underwent adrenalectomy, and R0 was the post-surgical status in 10 of them. At the end of the study period, 11 patients were alive. The median progression-free survival was 18 months +/- 7.86, and the median overall survival was 30 months +/- 19.8.

Conclusion:

In the analyzed patient population, outcomes of progression-free survival and overall survival were similar to that reported at centers with extensive experience in adrenal disease.

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