Recurrence-Free Survival Analysis in Locally Advanced Pheochromocytoma: First Appraisal - Aix-Marseille Université Access content directly
Journal Articles Journal of Clinical Endocrinology and Metabolism Year : 2021

Recurrence-Free Survival Analysis in Locally Advanced Pheochromocytoma: First Appraisal

Sophie Moog
Christine Docao
  • Function : Author
Laurence Amar
Julien Hadoux
  • Function : Author
Charlotte Lussey-Lepoutre
Françoise Borson-Chazot
Delphine Vezzosi
  • Function : Author
Delphine Drui
  • Function : Author
Sandrine Laboureau
  • Function : Author
Marie-Laure Raffin Sanson
  • Function : Author
Livia Lamartina
  • Function : Author
Peggy Pierre
  • Function : Author
Marie Batisse Ligner
  • Function : Author
Ségolène Hescot
  • Function : Author
Abir Al Ghuzlan
  • Function : Author
Karine Renaudin
  • Function : Author
Rosella Libé
  • Function : Author
Suzanne Laroche
  • Function : Author
Gabrielle Deniziaut
  • Function : Author
Anne-Paule Gimenez-Roqueplo
Arnaud Jannin
  • Function : Author
Sophie Leboulleux
Carole Guerin
  • Function : Author
Eric Baudin


Abstract Context The behavior of locally advanced pheochromocytoma (LAP) remains unknown. Objective We characterized the population with LAP and recurrence-free survival (RFS). Methods This retrospective multicentric study was run within the ENDOCAN-COMETE network and French Group of Endocrine Tumors (GTE) from 2003 to 2018, including patients from 11 French referral centers with LAP as defined by capsular invasion, vascular invasion, adipose tissue invasion, and/or positive locoregional lymph nodes at diagnosis without evidence of distant metastasis. The main outcome measure was recurrence, defined as tumor reappearance, including local site and/or distant metastasis. The primary endpoint was RFS analysis; secondary endpoints were characterization, overall survival (OS), and prognostic factors of recurrence. Results Among 950 patients, 90 (9%) exhibited LAP criteria and 55 met inclusion criteria (median age, 53 years; 61% males; 14% with germline mutation; 84% with catecholamine excess). LAP was defined by 31 (56%) capsular invasions, 27 (49%) fat invasions, 6 (11%) positive lymph nodes, and 22 (40%) vascular invasions. After median follow-up of 54 months (range, 6-180), 12 patients (22%) had recurrences and 3 (5%) died of metastatic disease. Median RFS was 115 months (range, 6-168). Recurrences were local in 2 patients, distant in 2, and both local and distant in 8 patients. Median OS of patients was not reached. Size above 6.5 cm (P = 0.019) and Ki-67 > 2% (P = 0.028) were identified as independent significant prognostic factors in multivariate analysis. Conclusion LAP represents 9% of pheochromocytoma’s population and has a metastatic behavior. This study paves the way for future pathological TNM classification.
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Dates and versions

hal-03662818 , version 1 (09-05-2022)



Sophie Moog, Frederic Castinetti, Christine Docao, Laurence Amar, Julien Hadoux, et al.. Recurrence-Free Survival Analysis in Locally Advanced Pheochromocytoma: First Appraisal. Journal of Clinical Endocrinology and Metabolism, 2021, 106 (9), pp.2726-2737. ⟨10.1210/clinem/dgab202⟩. ⟨hal-03662818⟩
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