One-Year Progression-Free Survival of Therapy-Naive Patients With Malignant Pheochromocytoma and Paraganglioma

Author:

Hescot Ségolène1,Leboulleux Sophie1,Amar Laurence2,Vezzosi Delphine3,Borget Isabelle4,Bournaud-Salinas Claire5,de la Fouchardiere Christelle5,Libé Rossella6,Do Cao Christine7,Niccoli Patricia8,Tabarin Antoine9,Raingeard Isabelle10,Chougnet Cécile1,Giraud Sophie5,Gimenez-Roqueplo Anne-Paule11,Young Jacques12,Borson-Chazot Francoise5,Bertherat Jérôme13,Wemeau Jean-Louis7,Bertagna Xavier13,Plouin Pierre-Francois2,Schlumberger Martin1,Baudin Eric1,

Affiliation:

1. Institut Gustave Roussy (S.H., S.L., C.C., M.S., E.B.), Service de Médecine Nucléaire et de Cancérologie Endocrinienne, and Université Paris-Sud, Villejuif F-94805, France

2. Assistance Publique-Hôpitaux de Paris (L.A., P.-F.P.), Hôpital Européen Georges Pompidou, Service d'Hypertension Artérielle, Paris F-75015, France

3. CHU Larrey (D.V.), Service d'Endocrinologie, Maladies Métaboliques et Nutrition, Toulouse F-31059, France

4. Institut Gustave Roussy (I.B.), Biostatistic and Epidemiology Service and Université Paris-Sud, France

5. Groupement Hospitalier Lyon-Est (C.B.S., C.d.l.F., S.G., F.B.-C.), Fédération d'Endocrinologie et Centre de Médecine Nucléaire, Bron F-69677, France

6. Coordinator of the COrtico-MEdullo Tumeurs Endocrines group (R.L.), Lille F-59037, France

7. CHRU de Lille (C.D.C., J.-L.W.), Clinique Endocrinologique Marc-Linquette, Lille F-59037, France

8. Assistance Publique-Hôpitaux de Marseille (P.N.), CHU la Timone, Service d'Endocrinologie, Marseille F-13385, France

9. CHU Bordeaux (A.T.), Service d'Endocrinologie, Diabétologie et Maladies Métaboliques, Pessac F-33600, France

10. Hôpital Lapeyronie (I.R.), Maladies Endocriniennes, Montpellier F-34285, France

11. Assistance Publique-Hôpitaux de Paris (A.-P.G.-R.), Hôpital Européen Georges Pompidou, Service de Génétique, Paris F-75015, France

12. Assistance Publique-Hôpitaux de Paris (J.Y.), Hôpital de Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Le Kremlin Bicêtre F-94275, France

13. Assistance Publique-Hôpitaux de Paris (J.B., X.B.), Hôpital Cochin, Service d'Endocrinologie, Paris F-75014, France

Abstract

Abstract Context: The natural history of malignant pheochromocytoma or paragangliomas (MPP) remain unknown. Objective: The primary aim of this study was to define progression-free survival at 1 year in therapy-naive patients with MPP. Secondary objectives were to characterize MPP and to look for prognostic parameters for progression at 1 year. Design and Setting: The files of MPP followed up between January 2001 and January 2011 in two French Endocrine Networks were retrospectively reviewed. Therapy-naive patients were enrolled. Main Outcome Measures: The main outcome was progression-free survival at 1 year in therapy-naive MPP patients according to Response Evaluation Criteria In Solid Tumors 1.1 criteria. Results: Ninety files (46 men, 44 women, mean age of 47.5 ± 15 years) were reviewed on site by one investigator. MPP characteristics were as follows: presence of an adrenal primary, a mitotic count exceeding 5 per high power field, hypertension, inherited disease, and presence of bone metastases in 50%, 22%, 60%, 49%, and 56% patients, respectively. Fifty-seven of the 90 patients with MPP (63%) were classified as therapy-naive. The median follow-up of these 57 patients was 2.4 years (range, 0.4–5.7). At 1 year, progression-free survival was 46% (CI 95: 33–59). Twenty-six of 30 (87%) patients with progression at 1 year had exhibited progressive disease at the first imaging workup performed after a median of 5.7 months. No prognostic parameter was identified. Conclusions: Half of the therapy-naive patients with MPP achieved stable disease at 1 year. In symptom-free patients with MPP, a wait-and-see antitumor policy seems appropriate as first line. Modality for a prospective follow-up is proposed.

Publisher

The Endocrine Society

Subject

Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,Endocrinology, Diabetes and Metabolism

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