Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment

Abstract

Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological, and imaging features before surgery and pathological examination after tumor removal. Most patients present with steroid hormone excess or abdominal mass effects, but 15% of patients with ACC is initially diagnosed incidentally. After the diagnosis, in order to assess the ACC prognosis and establish an adequate basis for treatment decisions different tools are proposed. The stage classification proposed by the European Network for the Study of Adrenal Tumors (ENSAT) is recommended. Pathology reports define the Weiss score, the resection status and the proliferative index, including the mitotic count and the Ki67 index. As far as the treatment is concerned, in case of tumor limited to the adrenal gland, the complete resection of the tumor is the first option. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Recently, the First International Randomized (FIRM-ACT) Trial in metastatic ACC reported the association between mitotane and etoposide/doxorubicin/cisplatin (EDP) as the new standard in first line treatment of ACC. In last years, new targeted therapies, including the IGF-1 receptor inhibitors, have been investigated, but their efficacy remains limited. Thus, new treatment concepts are urgently needed. The ongoing “omic approaches” and next-generation sequencing will improve our understanding of the pathogenesis and hopefully will lead to better therapies.

Adrenocortical Carcinoma Treated by CyberKnife

“…Adrenocortical carcinoma is a rare malignancy with an incidence of 0.7-2.0 cases/million habitants/year (1). The prognosis is very poor.…”

Cushing Syndrome Revealing an Adrenocortical Carcinoma

“…In fact, after having demonstrated hypercortisolism, the second step is to confirm whether it is ACTH dependent or not [4]. This process was not complete for our patient because the dosage of ACTH and dexamethasone braking tests were not realizable.…”

“…It is a very rare tumor in pediatric pathology, representing less than 0.2% of all pediatric tumors [1]. Its global incidence is 0.5 to 2 cases/million inhabitants [2] [3] [4]. This malignant tumor is most often secreting.…”

A mikro-RNS-ek patogenetikai és diagnosztikai szerepe mellékvesekéreg-carcinomában

“…A mellékvesekéreg daganatos elváltozásai gyakoriak, boncolási adatok alapján a népesség 1,4-6%-át érintik [1,2]. Bár ezek nagy része hormonálisan inaktív jóindulatú daganatként nem okoz tünetet, s csupán véletlensze-rűen, más betegség kapcsán végzett képalkotó vizsgálat során fedezik fel (incidentaloma) [3], a ritka hormonszekretáló adenomák súlyosan ronthatják a betegek élet-minőségét.…”