Strong smooth muscle differentiation is dependent on myocardin gene
amplification in most human retroperitoneal leiomyosarcomas.
Cancer Res. 2009 Mar 15;69(6):2269-78. Epub 2009 Mar 10.
Pérot G, Derré J, Coindre JM, Tirode F, Lucchesi C, Mariani O, Gibault
L, Guillou L, Terrier P, Aurias A.
Genetics and Biology of Cancers, Institut Curie, Paris, France.
Myocardin (MYOCD), a serum response factor (SRF) transcriptional
cofactor, is essential for cardiac and smooth muscle development and
differentiation. We show here by array-based comparative genomic
hybridization, fluorescence in situ hybridization, and expression
analysis approaches that MYOCD gene is highly amplified and
overexpressed in human retroperitoneal leiomyosarcomas (LMS), a very
aggressive well-differentiated tumor. MYOCD inactivation by shRNA in a
human LMS cell line with MYOCD locus amplification leads to a dramatic
decrease of smooth muscle differentiation and strongly reduces cell
migration. Moreover, forced MYOCD expression in three undifferentiated
sarcoma cell lines and in one liposarcoma cell line confers a strong
smooth muscle differentiation phenotype and increased migration
abilities. Collectively, these results show that human retroperitoneal
LMS differentiation is dependent on MYOCD
amplification/overexpression, suggesting that in these
well-differentiated LMS, differentiation could be a consequence of an
acquired genomic alteration. In this hypothesis, these tumors would
not necessarily derive from cells initially committed to smooth muscle
differentiation. These data also provide new insights on the cellular
origin of these sarcomas and on the complex connections between
oncogenesis and differentiation in mesenchymal tumors.
PMID: 19276386
:: LMS Chat ::
:: Leiomyosarcoma (LMS) Defined ::
Leiomyosarcoma (Gr. Leio- =smooth, myo- = muscle, sarcoma = fleshy growth) is a type of sarcoma which is a neoplasm of smooth muscle. (When a uterine neoplasm is benign, it is a leiomyoma.) Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, and skin. It is therefore possible for leiomyosarcomas to appear at any site in the body. It is however most commonly found in the stomach, small intestine and retroperitoneum.
Leiomyosarcoma is a very rare cancer. It makes up 5-10% of soft tissue sarcomas, which are in themselves rare cancers.
:: About Leiomyosarcoma ::
Leiomyosarcoma is a form of cancer that affects the smooth muscle of the body. It spreads through the blood stream and can affect the lungs, liver, blood vessels, or any other soft tissue in the body.
LMS is a type of sarcoma which is a neoplasm of smooth muscle. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in the uterus, stomach and intestines, walls of all blood vessels, and skin.
Leiomyosarcoma is a very rare cancer. It makes up 7% of soft tissue sarcomas; in all, LMS affects 4 out of 1,000,000 people.
Presently there is no cure. Remission can be attained, but this rare cancer can reappear at any time. Because of its rarity, few doctors know how to treat it and it attracts very little research.
Subscribe to:
Post Comments (Atom)
No comments:
Post a Comment