The role of surgical treatment in stage IV adrenocortical cancer

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer with a very poor prognosis. Surgery remains the best treatment modality for adrenocortical carcinoma in the early stages. The appropriate treatment for metastatic ACC is not well established, and the effectiveness of chemotherapy and radiotherapy, is not proven. Five-year survival for patients with stage IV tumors ranged from 0 to 17 %. The data regarding the effectiveness of surgery in the management of metastatic tumor remain scarce.
Aim of the study – to present our results of surgical treatment for metastatic ACC in children and to determine the risk factors of local relapse.
Materials and methods. The results of treatment of 10 patients with a median age of 5.8 (1.5–15.1) years with stage IV ACC were retrospectively analyzed. The tumors were hormone-producing in 6 of 10 cases. Four (40 %) patients experienced a Cushing syndrome. Primary tumor resection R1 and R0 was performed in 8 (80 %) and 5 (50 %) patients respectively. The median volume of resected tumor was 183 (3.6–1608) cm³. All patients received mitotane combined in 80 % with systemic chemotherapy consisted of etoposide, doxorubicin, cisplatin (EDP-M).
Results. Two (20 %) of 10 patients are alive with tumor in 14.1 and 28.8 months respectively. The median follow-up in the hole group was 21.8 (10.4–33.9) months and the median period to disease progression was 11.3 (6.4–17.9) months. The risk of local relapse in the first 8 months after surgery was 33.3 % vs. 100 % in R0 and R1/R2 groups respectively. The period from diagnosis to death from tumor progression was significantly longer in 7 patients with 2 and more EDP-M courses compared to 3 patients who received one EDP-M or no further treatment (24.9 vs. 14.4 months, p = 0.02). Two and five-year overall survive were 57.1 % and 0 % respectively.
Conclusion. The radical surgical resection (R0) significantly reduced the risk and incidence of local relapse in children with stage IV ACC. Adjuvant EDP-M therapy did not affect the unfavorable prognosis of the disease, but positively influenced life expectancy of patients with disseminated ACC.

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