One-year progression-free survival of therapy-naive patients with malignant pheochromocytoma and paraganglioma.
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Hescot Ségolène, Leboulleux Sophie, Amar Laurence, Vezzosi Delphine, Borget Isabelle, Bournaud-Salinas Claire, de la Fouchardiere Christelle, Libé Rossella, Do Cao Christine, Niccoli Patricia, Tabarin Antoine, Raingeard Isabelle, Chougnet Cécile, Giraud Sophie, Gimenez-Roqueplo Anne-Paule, Young Jacques, Borson-Chazot Francoise, Bertherat Jérôme, Wemeau Jean-Louis, Bertagna Xavier, Plouin Pierre-Francois, Schlumberger Martin, Baudin Eric, French group of Endocrine and Adrenal tumors (Groupe des Tumeurs Endocrines-REseau NAtional des Tumeurs ENdocrines and COrtico-MEdullo Tumeurs Endocrines networks)
Аннотация
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.