Adrenocortical carcinomas: surgical trends and results of a 253‐patient series from the French Association of Endocrine Surgeons study group

Abstract

Because of the rarity of adrenocortical carcinoma, survival rates and the prognosis for patients who have undergone operation are not well known. The purpose of the French Association of Endocrine Surgery was to evaluate these factors over an 18-year period. A trend study was associated to assess changes in the clinical and biochemical presentations as well as the surgical evolution. A total of 253 patients (158 women, 95 men) with a mean age of 47 years were included. Cushing syndrome was the main clinical presentation (30%), and hormonal studies revealed secreting tumors in 66% of the cases. Altogether, 72% (n = 182) of patients underwent resection for cure, and 41.5% (n = 105) of them had an extensive resection because of metastatic cancer. A lymphadenectomy was performed in 32.5% (n = 89) of the cases. The operative mortality was 5.5% (n = 14). Patients were given mitotane as adjuvant therapy in 53.8% of the cases (n = 135). The results of staging were stage I in 16 patients (6.3%), stage II (local disease) in 126 patients (49.8%), stage III (locoregional disease) in 57 patients (22.5%), and stage IV (metastases) in 54 patients (21.3%). Neither tumor staging nor the rate of curative surgery changed during the study period. More subcostal incisions were performed, and the use of mitotane increased significantly. The 5-year actuarial survival rates were 38% overall, 50% in the curative group, 66% for stage I, 58% for stage II, 24% for stage III, and 0% for stage IV. Multivariate analysis showed that mitotane benefited only the group of patients not operated on for cure. A better prognosis was found in patients operated on after 1988 (p = 0.04), in those with precursor-secreting tumors (p = 0.005), and in those at local stages of the disease (p = 0.0003). Thus mitotane benefited only patients not operated on for cure. Curative resection, precursor secretion, recent diagnosis, and local stage were favorably associated with survival.

Combination Chemotherapy in Advanced Adrenocortical Carcinoma

“…[3][4][5][6][7] Mitotane is the only drug approved for the treatment of adrenocortical carcinoma and is used both as adjuvant therapy and for advanced disease, [8][9][10][11][12][13][14] although its efficacy has never been shown in a randomized trial. The experience with other antineoplastic drugs for the treatment of this disease is even more limited.…”

Prevalence and natural history of adrenal incidentalomas

“…However, other investigators have reported similar staging of disease at diagnosis and survival rates between incidentally discovered adrenocortical carcinomas and non-incidental carcinomas (111 -114). Indeed, the incidental identification of asymptomatic adrenocortical incidentalomas seems to have led to only a very modest reduction in the number of patients who present with metastatic disease (113,115).…”

Single nucleotide polymorphism array profiling of adrenocortical tumors: evidence for an adenoma carcinoma sequence?

“…ACC is a highly aggressive tumor with poor prognosis and an overall survival rate of 5 years of 25-50% in most series [1,2,3,4,5,6]. The rarity of ACCs is a major limitation to our understanding of their pathophysiology.…”