ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 EP18 | DOI: 10.1530/endoabs.63.EP18

Characteristics, management and outcome of patients with adrenocortical carcinoma in a tertiary hospital: a retrospective study

Labrini Papanastasiou1, Theodosia Choreftaki2, George Zografos3, Chara Kapsali1, Athanasia Kalantzi1, Nikoleta Monastirioti1, Christos Gravvanis1, Spyridoula Glykofridi1, Ernestini Tyfoxilou1, Sofia Vlaxou1, Liana Charalampidou1, Nikos Voulgaris1, Athina Markou1, Irini Giagourta1, George Piaditis1 & Theodora Kounadi1


1Department of Endocrinology and Diabetes Center, General Hospital of Athens ‘G. Gennimatas’, Athens, Greece; 2Department of Pathology, General Hospital of Athens ‘G. Gennimatas’, Athens, Greece; 33rd Department of Surgery, General Hospital of Athens ‘G. Gennimatas’, Athens, Greece.


Adrenocortical carcinoma (ACC) is a rare, aggressive tumor with poor prognosis, affecting 0.5–2 cases/106population/year.

Objective: To analyse retrospectively characteristics, management and outcome of ACC patients, followed in our center between 2010–2018.

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.