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J Clin Endocrinol Metab. 2013 Oct;98(10):4006-12. doi: 10.1210/jc.2013-1907. Epub 2013 Jul 24.

One-year progression-free survival of therapy-naive patients with malignant pheochromocytoma and paraganglioma.

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1
Department of Nuclear Medicine and Endocrine Tumors, Institut Gustave Roussy and University Paris-Sud, 114 rue Edouard Vaillant, 94805 Villejuif Cedex, France. baudin@igr.fr.

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.

PMID:
23884775
DOI:
10.1210/jc.2013-1907
[Indexed for MEDLINE]
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