Are Peripheral Pure Pleomorphic Sarcomas with MDM2 Amplification Dedifferentiated Liposarcomas?
Sophie Le Guellec, Agnes Neuville, Frederic Chibon, Dominique Ranchere-Vince, Frederique Larousserie, Philippe Terrier, Jean-Michel Coindre. Institut Claudius Regaud, Toulouse, France; Institut Bergonié, Bordeaux, France; Centre Léon Bérard, Lyon, France; CHU Cochin, Paris, France; Institut Gustave Roussy, Paris, for the French Sarcoma Group, France
Background: Dedifferentiated liposarcoma (DDLPS) has been defined as a tumor composed of well-differentiated liposarcoma (WDLPS) associated with a nonlipogenic undifferentiated sarcoma and is genetically characterized by a 12q13-14 amplicon with MDM2 amplification. Some peripheral (extremities, trunk wall, head and neck) undifferentiated pleomorphic sarcomas without areas of WDLPS present a MDM2 amplification (PUPS-MDM2 pos).The purpose of this study is to address whether they are true DDLPS or not.
Design: The present study compared clinical data, histology, genomic profile (array-CGH) and follow-up (overall survival-OS, metastasis-free survival-MFS and local recurrence-free survival-LRFS) in PUPS-MDM2 pos and peripheral conventional (with areas of WDLPS) DDLPS (PC-DDLPS) patients retrieved from 1-the French sarcoma network RRePS (http://www.rreps.sarcomabcb.org) for systematic histological review (2010 and 2011) and 2-the Conticabase (http://www.conticabase.sarcomabcb.org)(1980 to 2011). We only considered patients with a resection of the primary tumor and with adequate sampling.
Results: We collected respectively 10 PUPS-MDM2 pos and 120 PC-DDLPS (which accounted for 25.3% of all DDLPS) in RRePS and 10 PUPS-MDM2 pos and 80 PC-DDLPS in the Conticabase. Follow–up was available for 20 PUPS-MDM2 pos and 80 PC-DDLPS. Clinical characteristics and follow-up are shown in Table. Array-CGH performed in 20 PUPS-MDM2 pos and in 20 PC-DDLPS showed a typical profile of DDLPS with a 12q13-14 amplicon.
|Morphological features of dedifferentiated component||Spindle/pleomorphic||15||40||0.194|
|Age at diagnosis (years)||Median (range)||77 (49-90)||67 (18-88)||0.006|
|Tumor size (cm)||Median (range)||9.75 (2.5-20)||13 (2.5-50)||0.077|
|Follow-up,years||Median (95%CI)||22.9 (3.9-NA)||28.8 (15.1-40.1)|