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Submitted on December 15, 2005
Accepted on April 24, 2006
Centre de Référence Maladies Rares de la Surrénale, Service des Maladies Endocriniennes et Métaboliques,, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; Service de Biostatistique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; INSERM U567 and CNRS UMR 8104, Institut Cochin, Paris, France; Service d'Orthopédie B, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; Service d'Anatomo-pathologie, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; Service de Radiologie A, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France; Service de Chirurgie Digestive et Endocrine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5, Paris, France
* To whom correspondence should be addressed. 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, setting: Descriptive and multivariate analysis of a cohort from a single endocrinology center.
Patients: 202 consecutive patients with ACC.
Results: The sex ratio (F/M) was 2.7. Mean age at diagnosis was 44 ± 16 yr (range 11 to 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 (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 o,p'DDD in the three months following 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 non invasive 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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