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

Centre de Référence Maladies Rares de la Surrénale, Service des Maladies Endocriniennes et Métaboliques (G.A., L.G., X.B., J.B.), Service de Biostatistique (J.C.), Service d’Orthopédie B (P.A.), Service d’Anatomo-pathologie (F.T.), Service de Radiologie A (P.L.), Service de Chirurgie Digestive et Endocrine (B.D.), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; and Institut National de la Santé et de la Recherche Médicale Unité 567 and Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104 (L.G., F.T., X.B., J.B.), Institut Cochin, Paris, France

Address all correspondence and requests for reprints to: Prof. Jérôme Bertherat, Service d’Endocrinologie, Hôpital Cochin, 27, rue du Faubourg Saint-Jacques, 75014, Paris, France. E-mail: jerome.bertherat{at}cch.aphp.fr.

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.

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