title: Andrenocortical carcinomas: twelve-year prospective experience.
abstract: Adrenocortical carcinoma (AC) is a rare tumor with poor prognosis. Twenty-two patients (14 F, 8 M; age 22 to 59 years; median, 43 years) with AC were evaluated prospectively in a single center: tumor stage was I-II in 12 cases and III-IV in 10. The overall survival in our cohort was 41.6 +/- 42 months; 16 subjects are still alive. Curative surgery was followed by longer survival than debulking or no surgery (p < 0.0001). The first relapse was highly predictive for further recurrences. Recurrent ACs were progressively more aggressive, and they occurred with variable but ever shorter intervals. At diagnosis, 14 patients (63.5%) presented with features of clear adrenocortical hyperactivity. Despite the absence of clinical signs of hormonal excess, all other patients presented some abnormalities of steroid secretion. The most common clinical finding was a recent diagnosis of moderate-to-severe hypertension (68%), poorly controlled by pharmacological treatment, often associated with multiple cardiovascular risk factors. High mitotic rate and undifferentiated polymorph cellular pattern were associated with worse prognosis. Response to treatments other than surgery (mitotane chemotherapy) was better in patients treated early after the first surgery. In conclusion, curative surgery was the most effective treatment. Monitoring arterial pressure, endocrine parameters, and metabolic parameters can be helpful for the early detection of AC recurrences.
authors.affiliation: Department of Molecular and Clinical Endocrinology and Oncology, Federico II University of Naples, Via S. Pansini 5, 80131 Naples, Italy. tauchman@unina.it
authors.forename: Libuse
authors.initials: L
authors.lastname: Tauchmanovà
authors.forename: Annamaria
authors.initials: A
authors.lastname: Colao
authors.forename: Luigi Antonio
authors.initials: LA
authors.lastname: Marzano
authors.forename: Lucianna
authors.initials: L
authors.lastname: Sparano
authors.forename: Luigi
authors.initials: L
authors.lastname: Camera
authors.forename: Annalisa
authors.initials: A
authors.lastname: Rossi
authors.forename: Giovanna
authors.initials: G
authors.lastname: Palmieri
authors.forename: Ettore
authors.initials: E
authors.lastname: Marzano
authors.forename: Marco
authors.initials: M
authors.lastname: Salvatore
authors.forename: Guido
authors.initials: G
authors.lastname: Pettinato
authors.forename: Gaetano
authors.initials: G
authors.lastname: Lombardi
authors.forename: Riccardo
authors.initials: R
authors.lastname: Rossi
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000175:diagnosis / Q000601:surgery*; D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013997:Time Factors; D016896:Treatment Outcome