EJE PRIZE 2014: Current and evolving treatment options in adrenocortical carcinoma: where do we stand and where do we want to go?

Abstract

Adrenocortical carcinoma (ACC) is not only a rare and heterogeneous disease but also one of the most aggressive endocrine tumors. Despite significant advances in the last decade, its pathogenesis is still only incompletely understood and overall therapeutic means are unsatisfactory. Herein, we provide our personal view of the currently available treatment options and suggest the following research efforts that we consider timely and necessary to improve therapy: i) for better outcome in localized ACCs, surgery should be restricted to experienced centers, which should then collaborate closely to address the key surgical questions (e.g. best approach and extent of surgery) in a multicenter manner. ii) For the development of better systemic therapies, it is crucial to elucidate the exact molecular mechanisms of action of mitotane. iii) A prospective trial is needed to address the role of cytotoxic drugs in the adjuvant setting in aggressive ACCs (e.g. mitotane vs mitotaneC cisplatin). iv) For metastatic ACCs, new regimens should be investigated as first-line therapy. v) Several other issues (e.g. the role of radiotherapy and salvage therapies) might be answered -at least in a first step -by large retrospective multicenter studies. In conclusion, although it is unrealistic to expect that the majority of ACCs can be cured within the next decade, international collaborative efforts (including multiple translational and clinical studies) should allow significant improvement of clinical outcome of this disease. To this end, it might be reasonable to expand the European Network for the Study of Adrenal Tumors (ENSAT) to a truly worldwide international network -INSAT.

Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

“…Adrenocortical carcinoma (ACC) is a rare and very aggressive endocrine malignancy [1] with an annual incidence of 0.7-2.0 cases per million of people [2]. The 5-year survival ranges from 81% for European Network for the Study of Adrenal Tumours (ENSAT) tumour stage I to 13% for ENSAT tumour stage IV [3], although there's an interindividual marked heterogeneity [4].…”

“…Several collaborative efforts have been made and much has been published [1,2] but the best strategy is still unclear and advancements are needed in order to improve diagnosis, therapy and clinical outcome [5]. Surgery is the only potential curative treatment [6] and mitotane remains the mainstay treatment after surgical removal of the tumour [7].…”

“…Surgery is the only potential curative treatment [6] and mitotane remains the mainstay treatment after surgical removal of the tumour [7]. The role of radiotherapy and salvage therapies are not completely established [1] and the diagnosis is still too late and the prognosis poor.…”

“…[1,8]), when to perform radiotherapy, the role of cytotoxic drugs and the best combination chemotherapy in advanced ACC. To address all these issues, there's a need for new translational studies, basic research and large multicentre randomized clinical trials to establish the best standards of care.…”

Case Report: Adrenocortical Carcinoma with a Solitary Fibrous Tumor

“…Complete surgical resection is the only potentially curative treatment [5] for patients with the localized disease; however, due to a recurrence rate of 50~70% after apparent radical surgery, there is a strong rationale for a concomitant systemic treatment [6].…”