Prognosis of Malignant Pheochromocytoma and Paraganglioma (MAPP-Prono Study): A European Network for the Study of Adrenal Tumors Retrospective Study

• Published in: The journal of clinical endocrinology and metabolism Vol. 104; no. 6; pp. 2367 - 2374
• Main Authors: Hescot, Segolene, Curras-Freixes, Maria, Deutschbein, Timo, van Berkel, Anouk, Vezzosi, Delphine, Amar, Laurence, de la Fouchardière, Christelle, Valdes, Nuria, Riccardi, Fernando, Do Cao, Christine, Bertherat, Jerome, Goichot, Bernard, Beuschlein, Felix, Drui, Delphine, Canu, Letizia, Niccoli, Patricia, Laboureau, Sandrine, Tabarin, Antoine, Leboulleux, Sophie, Calsina, Bruna, Libé, Rossella, Faggiano, Antongiulio, Schlumberger, Martin, Borson-Chazot, Francoise, Mannelli, Massimo, Gimenez-Roqueplo, Anne-Paule, Caron, Philippe, Timmers, Henri J L M, Fassnacht, Martin, Robledo, Mercedes, Borget, Isabelle, Baudin, Eric
• Format: Journal Article
• Language: English
• Published: Washington, DC Endocrine Society 01.06.2019; Oxford University Press
• Subjects: Adrenal Gland Neoplasms - genetics; Adrenal Gland Neoplasms - mortality; Adult; Aged; Analysis; Development and progression; Diagnosis; Female; Head and neck; Humans; Lymph nodes; Magnetic resonance imaging; Male; Malignancy; Medical prognosis; Metastases; Metastasis; Middle Aged; Multivariate analysis; Mutation; Neuroendocrine tumors; Paraganglioma; Paraganglioma - genetics; Paraganglioma - mortality; Pheochromocytoma; Pheochromocytoma - genetics; Pheochromocytoma - mortality; Prognosis; Retrospective Studies; Succinate Dehydrogenase - genetics; Survival; Tumors; France; Spain
• ISSN: 0021-972X; 1945-7197; 1945-7197
• DOI: 10.1210/jc.2018-01968

Abstract Background Malignant pheochromocytoma and paraganglioma (MPP) are characterized by prognostic heterogeneity. Our objective was to look for prognostic parameters of overall survival (OS) in MPP patients. Patients and Methods Retrospective multicenter study of MPP characterized by a neck-thoraco-abdomino-pelvic CT or MRI at the time of malignancy diagnosis in European centers between 1998 and 2010. Results One hundred sixty-nine patients from 18 European centers were included. Main characteristics of patients with MPP were: primary pheochromocytoma in 53% of patients; tumor- or hormone-related symptoms in 57% or 58% of cases; positive plasma or urine hormones in 81% of patients; identification of a mutation in SDHB in 42% of cases. Metastatic sites included bone (64%), lymph node (40%), lung (29%), and liver (26%); mean time between initial and malignancy diagnosis was 43 months (range, 0 to 614). Median follow-up was 68 months and median survival 6.7 years. Using univariate analysis, better survival was associated with head and neck paraganglioma, age <40 years, metanephrines less than fivefold the upper limits of the normal range, and low proliferative index. In multivariate analysis, hypersecretion [hazard ratio 3.02 (1.65 to 5.55); P = 0.0004] was identified as an independent significant prognostic factor of worst OS. Conclusions Our results do not confirm SDHB mutations as a major prognostic parameter in MPP and suggest additional key molecular events involved in MPP tumor progression. Aside from SDHB mutation, the biology of aggressive MPP remains to be understood. Retrospective study that identifies hormonal hypersecretion as an independent significant prognostic factor of worst overall survival, whereas SDHB mutations have no prognostic impact.