Details

Autor(en) / Beteiligte

• Abiven, Gwenaelle
• Coste, Joel
• Groussin, Lionel
• Anract, Philippe
• Tissier, Frédérique
• Legmann, Paul
• Dousset, Bertrand
• Bertagna, Xavier
• Bertherat, Jérôme

Titel

Clinical and Biological Features in the Prognosis of Adrenocortical Cancer: Poor Outcome of Cortisol-Secreting Tumors in a Series of 202 Consecutive Patients

Ist Teil von

• The journal of clinical endocrinology and metabolism, 2006-07, Vol.91 (7), p.2650-2655

Ort / Verlag

Bethesda, MD: Endocrine Society

Erscheinungsjahr

2006

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• Context: Adrenocortical carcinomas (ACC) are rare tumors with a poor prognosis. Few reports concerning large and homogeneous series are available. Objective: We aimed to determine the clinical characteristics and outcome of ACC and to identify prognostic factors. Design and Setting: This study is a descriptive and multivariate analysis of a cohort from a single endocrinology center. Patients: A total of 202 consecutive patients with ACC were studied. Results: The sex ratio (female to male) was 2.7. Mean age at diagnosis was 44 ± 16 yr (range, 11–88 yr). We found that 154 patients (76%) had hypersecreting tumors [mostly cortisol and androgens (47%), cortisol alone (27%), or androgens alone (6%)] and 43 patients (21%) had metastases at diagnosis. At initial staging or during follow-up, 85 patients (42%) had liver metastases, 79 patients (39%) had lung metastases, and 20 patients had bone metastases (10%). The survival rate was 37% at 5 yr. Multivariate analysis identified the following independent prognostic factors associated with shorter survival: older age at diagnosis [hazard ratio (HR), 1.03; P < 0.0001], initial MacFarlane extension stages 3 (HR, 4.42; P = 0.005) and 4 (HR, 7.93; P < 0.0001), and cortisol hypersecretion (HR, 3.90; P < 0.0001). Treatment with 1,1-dichlorodiphenildichloroethane (o,p’DDD) in the 3 months after surgery increased the survival rate of patients with cortisol-secreting tumors (HR, 0.40; P = 0.04). Conclusion: This study highlights the better prognosis of ACC diagnosed at a noninvasive local stage, the particularly poor prognosis of patients with cortisol-secreting tumors, and the beneficial effect of o,p’DDD therapy in this subgroup of patients.