title: Adrenocortical cancer (ACC) - literature overview and own experience.
abstract: Adrenocortical carcinoma (ACC) is a malignant endocrine tumour. The rarity of the disease has stymied therapeutic development. Age distribution shows two peaks: the first and fifth decades of life, with children and women more frequently affected. Although 60-70% of ACCs are biochemically found to overproduce hormones, it is not clinically apparent in many cases. If present, endocrine symptoms include signs of hypercortisolaemia, virilisation or gynaecomastia. ACC carries a poor prognosis, and a cure can be achieved only by complete surgical resection. Mitotane is used both as an adjuvant treatment and also in non-operative patients. The role of radio- and chemotherapy is still controversial. The post-operative disease free survival is low and oscillates around 30% due to high tumour recurrence rate. The diagnosis is based on tumour histological assessment with the use of the Weiss score, however urinary steroid profiling (if available) can serve to differentiate between ACC and other adrenal tumours. Conventional prognostic markers in ACC include stage and grade of disease, and, as currently reported, the presence of hypercortisolaemia. Molecular analysis has had a significant impact on the understanding of the pathogenetic mechanism of ACC development and the evaluation of prognostic and predictive markers, among which alterations of the IGF system, the Wnt pathway, p53 and molecules involved in cancer cell invasion properties and angiogenesis seem to be very promising. We here summarise our own experience related to the management of ACC and present a literature overview. We have not aimed to include a detailed summary of the molecular alterations biology described in ACC, as this has already been addressed in other papers.
authors.affiliation: *Both co-authors contributed to the manuscript equally and both should be considered as 1st co-author Department of Endocrinology and Internal Medicine, Medical University of Gdansk, Gdansk, Poland Departments of Medicine and Endocrinology, King's College Hospital, London, UK Richard Dimbleby Department of Cancer Research, King's College London, Guy's Medical School Campus, London, UK. ddw@gumed.edu.pl.
authors.forename: Dorota
authors.initials: D
authors.lastname: Dworakowska
authors.forename: Agata
authors.initials: A
authors.lastname: Drabarek
authors.forename: Ingrid
authors.initials: I
authors.lastname: Wenzel
authors.forename: Anna
authors.initials: A
authors.lastname: Babińska
authors.forename: Renata
authors.initials: R
authors.lastname: Świątkowska-Stodulska
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Sworczak
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000628:therapy*; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D012307:Risk Factors
chemical_list: D018931:Antineoplastic Agents, Hormonal