PMID- 24525499
OWN - NLM
STAT- MEDLINE
DCOM- 20140410
LR  - 20161125
IS  - 1532-0979 (Electronic)
IS  - 0147-5185 (Linking)
VI  - 38
IP  - 3
DP  - 2014 Mar
TI  - Are peripheral purely undifferentiated pleomorphic sarcomas with MDM2 
      amplification dedifferentiated liposarcomas?
PG  - 293-304
LID - 10.1097/PAS.0000000000000131 [doi]
AB  - Dedifferentiated liposarcoma (DDLPS) has been defined as a tumor composed of 
      well-differentiated liposarcoma associated with a nonlipogenic undifferentiated 
      sarcoma and is genetically characterized by a 12q13-15 amplicon with MDM2 
      amplification. Some peripheral (extremities, trunk wall, head/neck) 
      undifferentiated pleomorphic sarcomas (UPS) without areas of well-differentiated 
      liposarcoma present an MDM2 amplification. We addressed whether they are true 
      DDLPS or not. We compared the clinical data, histologic data, MDM2 status 
      (immunohistochemistry [IHC], fluorescence in situ hybridization [FISH]), genomic 
      profile (array comparative genomic hybridization), and follow-up of 19 patients 
      with peripheral UPS with MDM2 amplification and 62 with peripheral conventional 
      DDLPS retrieved from the French sarcoma network (RRePS) and the Conticabase 
      (Connective Tissue Cancer Network database). For a control cohort, we described 
      153 patients from the Conticabase, with peripheral UPS without expression of MDM2 
      by IHC. By IHC, tumor cells were positive for MDM2 in 59 conventional DDLPS and 
      in all UPS with MDM2 amplification. FISH analysis and/or quantitative polymerase 
      chain reaction showed amplification of MDM2 in 54 conventional DDLPS and in all 
      UPS with MDM2 amplification. The 2-year overall survival rates of UPS with MDM2 
      amplification, conventional DDLPS, and UPS without expression of MDM2 were 93.3%, 
      90.7%, and 73.9%, respectively. Such similarities in the clinical 
      characteristics, morphology, genomic profile, and follow-up of peripheral UPS 
      with MDM2 amplification and peripheral conventional DDLPS strongly suggest that 
      peripheral UPS with MDM2 amplification are in fact DDLPS. Faced with histologic 
      diagnosis of UPS, a systematic IHC evaluation of MDM2 allows a selection of cases 
      for FISH analysis permitting the diagnosis of DDLPS.
FAU - Le Guellec, Sophie
AU  - Le Guellec S
AD  - Departments of *Pathology double daggerBiostatistics, Institut Claudius Regaud, Toulouse 
      daggerDepartment of Pathology and INSERM U916, Institut Bergonie **University Bordeaux 
      Segalen, Bordeaux  section signDepartment of Pathology, Centre Leon Berard, Lyon  parallelDepartment 
      of Pathology, Centre Oscar Lambret, Lille  paragraph signDepartment of Pathology, Hopital 
      Cochin, Assistance Publique-Hopitaux de Paris, Universite Paris Descartes, 
      Sorbonne Paris Cite #Department of Pathology, Institut Gustave Roussy, Paris, 
      France.
FAU - Chibon, Frederic
AU  - Chibon F
FAU - Ouali, Monia
AU  - Ouali M
FAU - Perot, Gaelle
AU  - Perot G
FAU - Decouvelaere, Anne-Valerie
AU  - Decouvelaere AV
FAU - Robin, Yves-Marie
AU  - Robin YM
FAU - Larousserie, Frederique
AU  - Larousserie F
FAU - Terrier, Philippe
AU  - Terrier P
FAU - Coindre, Jean-Michel
AU  - Coindre JM
FAU - Neuville, Agnes
AU  - Neuville A
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
RN  - 0 (Biomarkers, Tumor)
RN  - EC 2.3.2.27 (MDM2 protein, human)
RN  - EC 2.3.2.27 (Proto-Oncogene Proteins c-mdm2)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Biomarkers, Tumor/*analysis/genetics
MH  - *Cell Dedifferentiation
MH  - *Cell Differentiation
MH  - Diagnosis, Differential
MH  - Disease Progression
MH  - Disease-Free Survival
MH  - Female
MH  - *Gene Amplification
MH  - Gene Expression Profiling
MH  - Genetic Predisposition to Disease
MH  - Genetic Testing/methods
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Kaplan-Meier Estimate
MH  - Liposarcoma/*chemistry/classification/genetics/mortality/pathology
MH  - Male
MH  - Middle Aged
MH  - Phenotype
MH  - Predictive Value of Tests
MH  - Proto-Oncogene Proteins c-mdm2/*analysis/genetics
MH  - Retrospective Studies
MH  - Sarcoma/*chemistry/classification/genetics/mortality/pathology
MH  - Terminology as Topic
MH  - Time Factors
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2014/02/15 06:00
MHDA- 2014/04/11 06:00
CRDT- 2014/02/15 06:00
PHST- 2014/02/15 06:00 [entrez]
PHST- 2014/02/15 06:00 [pubmed]
PHST- 2014/04/11 06:00 [medline]
AID - 00000478-201403000-00002 [pii]
AID - 10.1097/PAS.0000000000000131 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 2014 Mar;38(3):293-304. doi: 10.1097/PAS.0000000000000131.