Abstract
BackgroundAdrenocortical carcinoma (ACC) is a rare malignant disease with a poor prognosis. Our aims were to study survival and to explore prognostic markers.MethodsWe retrospectively investigated the medical records of all 50 ACC patients at a single centre diagnosed between 1985 and 2012 and followed them up until 31/12/2014.ResultsOf this cohort, twenty six (52 %) were females. Adrenalectomy was performed in 48 patients (96 %), and twenty seven (54 %) were treated with adjuvant cytotoxic agents. The tumor sizes ranged from 6 to 20 cm. Overall survival time was 5.5 years (0.3–19.8), the two and five-year survival was 64 and 40 %, respectively. In ENSAT stage II 25/48 patients had a median survival of 7.0 years (0.7–15.5), in stage III 8/48 this was 1.9 (0.4 – 19.8), and in stage IV 15/48 it was 1.2 (0.3–3.6) years. Seventeen patients (34 %) were still alive at the end of 2014. The total follow-up time was 8.4 (0.3–19.8) years. Cell proliferation measured with Ki-67 had a median value of 15 % (2–80) and the urinary steroid profile was clearly pathologic in 29 of 43 (67 %) tested patients. The proliferation index did not significantly predict mortality (Ki-67 ≤ 10 vs. >10 %, 9.0 vs. 3.2 years, P = 0.0833), but resection margins did (R1 vs. R2, P = 0.0066; R0 vs. R2, P < 0.0001). The urinary steroid profile did not predict mortality (normal vs. pathologic urine profile: median survival 6.6 vs. 3.3 years, P = 0.261).ConclusionsThe prognosis was generally poor and macroscopically positive resection margins resulted in a worse prognosis. However, some patients were still alive many years following primary surgery with no sign of residual disease.
Highlights
Adrenocortical carcinoma (ACC) is a rare malignant disease with a poor prognosis
All patients had ACC diagnosed based on a combination of tumour size, clinical status, endocrine evaluation, radiology, histopathology, metastases at diagnosis or at follow-up and Urinary steroid profile (USP)
Eight patients (16 %) survived 10 years or more. In this cohort of patients with ACC diagnosed during three decades we found a generally poor prognosis, but some were still alive without any residual disease after
Summary
Our aims were to study survival and to explore prognostic markers. The annual incidence has been estimated to be between 0.5 and 2 cases per million people [1]. It typically occurs during late middle age, but onset can occur at a younger age. Close follow-up is mandatory with repeated radiology, and in case of endocrine hypersecretion of blood and urine markers. In recurrent disease repeated surgical resection of loco-regional disease is an option in selected patients [6]. The best prognostic factor after repeated surgery was time to first recurrence and radical surgery [7]
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