The impact of patient-, disease-, and treatment-related factors on survival in patients with adrenocortical carcinoma

Introduction: Adrenal cortical carcinoma (ACC) is a rare and aggressive endocrine tumour. Most present with advanced disease and have poor prognosis. Optimal treatment includes complete surgical resection. There is limited evidence for the efficacy of chemotherapy and radiation at different stages in this disease. There remain many inconsistencies with respect to diagnosis and workup. There is a lack of uniform guideline recommendations and consensus data. Methods: We performed a retrospective chart review of all patients at London Health Sciences Centre between 1990 and 2015 using ICD coding. All paper and electronic charts were reviewed and data was collected. Statistical analysis and survival curves were performed. Results: A total of 29 patients were included in our study. Median age was 55 years (interquartile range [IQR] 45-63); 14 (48%) were male and 15 (52%) were female. Approximately half (14 or 48%) of our patients presented symptomatically. Almost half (41%) of tumours were metabolically active, producing hormones. Most (88%) underwent surgical intervention. Surgical margin status was available in about half of patients and lymphadenectomy was performed in a third (n=8) of open adrenalectomy patients. A third received mitotane treatment (8 [73%] adjuvant and 3 [27%] palliative) and a third of patients received radiation. Two- and five-year median overall survival was 53% and 27%, respectively. Conclusions: ACC is a rare and aggressive tumour. This is the largest Canadian series reported to the best of our knowledge. Limited data for guidelines exists and treatment and workup patterns are inconsistent. Collaborative randomized and prospective studies on a global basis are needed.