Actual 10‐year survivors following resection of adrenocortical carcinoma

Abstract

Background Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options beyond surgical resection. The characteristics of actual long-term survivors following surgical resection for ACC have not been previously reported. Method Patients who underwent resection for ACC at one of 13 academic institutions participating in the US Adrenocortical Carcinoma Group from 1993 to 2014 were analyzed. Patients were stratified into four groups: early mortality (died within 2 years), late mortality (died within 2–5 years), actual 5-year survivor (survived at least 5 years), and actual 10-year survivor (survived at least 10 years). Patients with less than 5 years of follow-up were excluded. Results Among the 180 patients available for analysis, there were 49 actual 5-year survivors (27%) and 12 actual 10-year survivors (7%). Patients who experienced early mortality had higher rates of cortisol-secreting tumors, nodal metastasis, synchronous distant metastasis, and R1 or R2 resections (all P < 0.05). The need for multi-visceral resection, perioperative blood transfusion, and adjuvant therapy correlated with early mortality. However, nodal involvement, distant metastasis, and R1 resection did not preclude patients from becoming actual 10-year survivors. Ten of twelve actual 10-year survivors were women, and of the seven 10-year survivors who experienced disease recurrence, five had undergone repeat surgery to resect the recurrence. Conclusion Surgery for ACC can offer a 1 in 4 chance of actual 5-year survival and a 1 in 15 chance of actual 10-year survival. Long-term survival was often achieved with repeat resection for local or distant recurrence, further underscoring the important role of surgery in managing patients with ACC.

Treatment and management of adrenal cancer in a specialized Australian endocrine surgical unit: approaches, outcomes and lessons learnt

“…Our 5‐ and 10‐year survival rates of 52% and 24% are comparable to other series where 5‐year survivals ranged from 35% to 63% and 10‐year survival rates ranged between 7% and 38% . Factors associated with clear RFS and overall survival in our cohort are ENSAT stage and Ki‐67 level.…”

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

“…Thirty-five studies reporting on risk factors for recurrence and/or mortality, and that included more than 100 patients with histologically proven ACC, were analyzed (1,8,20,25,26,29,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82) (see Appendix 2 for details of studies included, and Appendix 3 for an overview of all prognostic factors studied). The threshold of 100 cases was defined upfront as with n = 100 and an expected number of deaths of 50, statistical power was considered sufficient.…”

Phytochemical analysis and biological activities of in vitro cultured Nidularium procerum, a bromeliad vulnerable to extinction

“…Until now, there have been only a few studies in the biological activity of N. procerum and none of them included the chemical compounds related to it, nor their potential as antitumor agents. Adrenocortical carcinoma is a rare and aggressive neoplasm with pour prognosis 88,89 , in which most patients diagnosed with advanced disease had a median survival time of less than 12 months and a 5-year survival rate of less than 15% among patients with metastatic disease 90 . In this scenario, the biological activity reported for N. procerum shows potential for the development of alternative treatments against adrenocortical carcinoma, which needs to be further explored through isolation and/or microencapsulation of bioactive compounds.…”