Adrenocortical carcinoma (ACC) is a rare, aggressive tumor with poor prognosis, affecting 0.52 cases/106population/year.
Objective: To analyse retrospectively characteristics, management and outcome of ACC patients, followed in our center between 20102018.
Material and methods: The medical records related to diagnosis, treatment and follow-up of nine patients of 59.2±13.8 years with histological diagnosis of ACC (Weiss score >3) were considered.
Results: At diagnosis, 4 patients suffered from muscle weakness and hypokalemia, 1 from abdominal pain, 1 from hirsutism and menstrual disturbances, 1 had severe hypertension and 2 were asymptomatic. Eight patients were functioning: 6 with hypercortisolism alone or in combination with either hyperandrogenism or hyperaldosteronism, 1 with isolated hyperaldosteronism and 1 with isolated hyperandrogenism. Mean adrenal tumor size was 7.96±4.43, Weiss score 5.7±1.9, Ki67 16.9±7.04% and mitotic count 18.4±14.5/50 HPFs. According to ENSAT staging: 2 patients were in stage IV, 5 in stage III, and 2 in stage II. Median follow-up was 27months. All patients underwent surgery: 5 had a complete resection and 3 patients underwent re-resection after recurrence. Mitotane was administrated in 8 out of 9 patients either as adjuvant (4) or as palliative (4) treatment. Simultaneous chemotherapy (EDP) was administrated in 1 patient. Median treatment duration was 20 months. Three patients (stage III or IV, Weiss score 8±1, Ki67 25±5, mitotic count 35±8.6 with treatment duration of 14.3±18.7 months) had progressive disease (PD); 2 (stage III or IV, Weiss score 4, Ki67 12.5±3.5, mitotic count 5.5±2.1 with treatment duration of 54.5±57.2 months) had complete response (CR); and 3 who received adjuvant treatment (stage II or III, Weiss score 4.6±0.57, Ki67 14±3.6, mitotic count 15.5±8.6 with treatment duration of 37±35.8 months) were with not evidence of disease. Patients with PD had higher Weiss score, Ki67 and mitotic count than those with no evidence of disease or with CR (P<0.05 in all comparisons). Therapeutic mitotane levels were achieved in 67% of patients with a daily dose of 5±1.4 gr within 6 months. Adrenal insufficiency occurred in all patients, hypothyroidism in 75% of patients. Serious adverse events (grade 3 and 4) occurred in 62% of patients. Two out of 3 patients who died discontinued treatment in<6 months because of serious adverse events.
Conclusions: In our center most ACC were functioning and diagnosed in advanced stages. Histological indices seem to be prognostics factors for the patients outcome. Mitotane was a beneficial treatment.
18 - 21 May 2019
European Society of Endocrinology