Locally advanced pheochromocytoma (LAP) behavior remains unknown.To characterize this population and its recurrence-free survival (RFS).A retrospective multicentric study run within the ENDOCAN-COMETE network and GTE from 2003 to 2018.11 French Referral centers.Patients 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.Recurrence was defined as the reappearance of the tumor, including local site and/or distant metastasis. The primary endpoint was RFS analysis. The secondary endpoints were characterization, overall survival (OS) and prognostic factors of recurrence.Among 950 patients, 90 exhibited LAP criteria (9%). 55 met the inclusion criteria (median age: 53 years-old, 61% males, 14% with a germline mutation, 84% with a 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 a median follow-up of 54 months (range, 6-180), 12 patients (22%) had recurrences and 3 (5%) died of a metastatic disease. Median RFS was 115 months (range, 6-168). The 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 6.5cm (p=0.019) and Ki-67>2% (p=0.028) were identified as independent significant prognostic factors in multivariate analysis.LAP represents 9% of pheochromocytoma's population and is characterized by a metastatic behavior. This study paved the way of a future pathological TNM classification.
Sophie Moog, Frédéric Castinetti, Christine DoCao, Laurence Amar, Julien Hadoux, Charlotte Lussey-Lepoutre, Françoise Borson-Chazot, Delphine Vezzosi, Delphine Drui, Sandrine Laboureau, Marie-Laure Raffin Sanson, Livia Lamartina, Peggy Pierre, Marie Batisse Ligner, Ségoléne Hescot, Abir Al Ghuzlan, Karine Renaudin, Rosella Libé, Suzanne Laroche, Gabrielle Deniziaut, Anne-Paule Gimenez-Roqueplo, Arnaud Jannin, Sophie Leboulleux, Carole Guerin, Matthieu Faron, Eric Baudin