Document Detail


Dedifferentiated liposarcomas with divergent myosarcomatous differentiation developed in the internal trunk: a study of 27 cases and comparison to conventional dedifferentiated liposarcomas and leiomyosarcomas.
MedLine Citation:
PMID:  17895758     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Dedifferentiated liposarcoma (DLPS) is one of the most frequent sarcomas of the retroperitoneum and represents most undifferentiated sarcomas of the internal trunk. In about 5% cases, the dedifferentiated component is an heterologous sarcoma such as leiomyosarcoma or rhabdomyosarcoma. We reviewed a series of 65 sarcomas with a myogenic differentiation developed in the internal trunk for which initial diagnoses were leiomyosarcoma (37), rhabdomyosarcoma (6), malignant mesenchymoma (6), and DLPS (16). Immunostainings for MDM2, CDK4, alpha smooth actin, desmin, caldesmon, myogenin, c-kit, and progesterone receptor were performed. In 48 cases, the amplification status of MDM2 and CDK4 could be evaluated with quantitative polymerase chain reaction on paraffin-embedded tissues extracted DNAs. After review of the cases, final diagnoses were leiomyosarcoma (35), rhabdomyosarcomatous (20) or leiomyosarcomatous (7) DLPS, probable DLPS (2), and malignant mesenchymoma (1). DLPS were bigger tumors (median: 18.2 cm) than leiomyosarcomas (median: 12 cm). They had a lower 5-year recurrence-free survival than leiomyosarcomas (45% vs. 71%) but a higher 5-year metastasis-free survival (73% vs. 39%). There was no significant difference in overall survival (57% vs. 34%). Outcome of patients with a DLPS with a myosarcomatous component did not differ from conventional DLPS. In conclusion, most sarcomas with a rhabdomyosarcomatous differentiation occurring in the internal trunk of adults are DLPS. Moreover, DLPS with a myogenic component have a low metastatic potential, similar to conventional DLPS and significantly lower to the metastatic potential of leiomyosarcomas.
Authors:
Matthieu Bui Nguyen Binh; Louis Guillou; Isabelle Hostein; Marie Christine Château; Francoise Collin; Alain Aurias; Bui Nguyen Binh; Eberhard Stoeckle; Jean-Michel Coindre
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of surgical pathology     Volume:  31     ISSN:  0147-5185     ISO Abbreviation:  Am. J. Surg. Pathol.     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-09-26     Completed Date:  2007-11-01     Revised Date:  2012-06-25    
Medline Journal Info:
Nlm Unique ID:  7707904     Medline TA:  Am J Surg Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1557-66     Citation Subset:  IM    
Affiliation:
Department of Pathology, Institut Bergonié and Université Victor Segalen, Bordeaux, France.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Aged, 80 and over
Cyclin-Dependent Kinase 4 / genetics,  metabolism
DNA, Neoplasm / analysis
Female
Humans
Immunoenzyme Techniques
Leiomyosarcoma / chemistry,  genetics,  mortality,  pathology*
Liposarcoma / chemistry,  genetics,  mortality,  pathology*
Male
Middle Aged
Nucleic Acid Amplification Techniques
Proto-Oncogene Proteins c-mdm2 / genetics,  metabolism
Retroperitoneal Neoplasms / chemistry,  genetics,  mortality,  pathology*
Rhabdomyosarcoma / chemistry,  genetics,  mortality,  pathology*
Soft Tissue Neoplasms / chemistry,  genetics,  mortality,  pathology*
Tumor Markers, Biological / analysis
Chemical
Reg. No./Substance:
0/DNA, Neoplasm; 0/Tumor Markers, Biological; EC 2.7.11.22/CDK4 protein, human; EC 2.7.11.22/Cyclin-Dependent Kinase 4; EC 6.3.2.19/MDM2 protein, human; EC 6.3.2.19/Proto-Oncogene Proteins c-mdm2

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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