Abstract
Context: Adrenocortical carcinoma (ACC) is a rare tumor with an incidence of 0.7-2 per million. Based on the ENSAT staging classification, tumor stage is the most important prognostic factor; the presence of lymph nodes involvement and metastases is an indicator of poor prognosis. Absence of any local or distant tumor invasion represents an early stage disease and is classified based on tumor size of <5 cm (stage I) or >5 cm (stage II). Advanced disease is confirmed if there is tumoral invasion, either locally in the surrounding tissues/nodes (stage III) or in other organs/vascular structures (stage IV).
Objective: To describe patient characteristics, staging and modes of presentation at initial diagnosis in our cohort of ACC patients.
Methods: We retrospectively reviewed paper and electronic charts of patients with pathology-confirmed ACCs who were treated at our referral center from 1995 to May 2019.
Results: One hundred four patients were diagnosed with ACC: 28 were men (26.9%) and 76 (73.1%) were women and median age was 51 years. The overall modes of presentation were hormonal hypersecretion (40.4%), mass-related symptoms (36.5%), incidentalomas (17.3%) and unknown (1.9%). Hormonal profile was available for 71 tumors: 67,6 % were secreting [androgen and cortisol co-secretion (39.4%), cortisol only (28.2%)] and 18,3% were non-secreting. At initial diagnosis, sixty-four patients (61.5%) had tumors >10 cm including 32.7% between 10-14.9 cm (n:34), 19.2% were 15-20 cm (n:20) and 9.6% were >20cm (n:10). Initial ENSAT stages were I (6.7%), II (17.3%), III (28.8%) and IV (44.2%) and unknown (2,9%). The age repartition was similar for most patients (median ~50 yo) regardless of disease stage or tumor size except in the subgroup of very large tumors (>20 cm) for which the median age was 40 yo. The mode of presentation at initial diagnosis varied at various stages. Incidentaloma was a frequent mode of presentation of earlier ACC stages; Stage 1: 3/7 (42,9%), stage II: 7/18 (38,9%), stage III: 4/30 (13,3%) and stage IV: 4/46 (8,7%). Hormonal excess symptoms led to ACC diagnosis less frequently in early stages (stages I and II) (24%) than in later stages (stage III and IV) (47,3%), while the hormonal work up showed high prevalence of secreting tumors in both groups (58,8% and 88,7%). Mass-related initial symptoms were similar in both groups 36% vs 39%.
Conclusions: In our cohort, 61.5% of ACC tumors were larger than 10 cm at initial diagnosis. Seventy-three percent of ACC patients had an advanced ENSAT stage III or IV disease which is associated with a 5 years survival of less than 50%. Incidentalomas is a frequent mode of presentation in stages I and II, while clinical hormonal excess symptoms were more frequent in later stages III and IV. Early stage diagnosis presents a difficult challenge in ACC and new biomarkers are needed to improve the odds against this deadly cancer.
Considerações acerca dos critérios de Identidade Intensional e Extensional na Conceitografia de Frege/Considerations on extensional and intensional identity criteria in Frege’s Begriffsschrift