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Soft Tissue Tumors: Soft Tissue Leiomyosarcoma

Identity

Note Soft Tissue Leiomyosarcoma is a relatively rare malignant tumor. It may be difficult to be distinguished from gastrointestinal stromal tumors and Schwann cell neoplasms. To make a correct identification of soft tissue leiomyosarcoma, immunostaining with several smooth muscle differentiation markers (actin, calponin and desmin), and negative staining results with S100 (to rule out Schwann cell neoplasm), c-kit and CD34 (to rule out gastrointestinal stromal tumors) is needed.

Clinics and Pathology

Clinics The annual new cases in the U.S. are over 6,000. The five year survival rate after diagnosis is about 50%.
 
Phylogenetic tree of leimyosarcomas and normal smooth muscle. Expression levels of 92 cDNA sequences from table III through V were analyzed for 11 leiomyosarcoma tissues. Experiment cluster analysis was performed using Michael Eisen's cluster tool and tree view. Data input and normalization of individual experiments were performed in GeneSpringTM 4.2 before imported into cluster tool. Red indicates high expression level, while green for low, black for no expression. The cutoff expression level for non-expressor was set at 200 arbitary units. Pathological grade (G) for each tumor is indicated, so is metastasis (r) if it is present.
Genes Soft tissue leiomyosarcoma was classified based on salient gene expression characteristics. Three types of leiomyosarcoma were proposed: 1) "Simplification" of gene expression in leiomyosarcoma, characterized by dramatic down regulation of large number of genes; 2) "Inflammation related" gene expression, characterized by the prominent presence of lymphocyte specific genes in the analysis; and 3) "neural" gene expression, characterized by neuronal gene expression. Among these subtypes, simplification gene expression is associated with the poorest prognosis, while inflammation related one the best.
Prognosis Local recurrent tumor, positive surgical margins, >50 years age, >20 mitoses per high power field are adversely associated with survival.

Bibliography

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Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities.
Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF
Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1996 ; 14 (5) : 1679-1689.
PMID 8622088
 
Gene expression analysis of human soft tissue leiomyosarcomas.
Ren B, Yu YP, Jing L, Liu L, Michalopoulos GK, Luo JH, Rao UN
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Contributor(s)

Written12-2004Jian-Hua Luo
Gene Array Laboratory, Univeristy of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA

Citation

This paper should be referenced as such :
Luo JH . Soft Tissue Tumors: Soft Tissue Leiomyosarcoma. Atlas Genet Cytogenet Oncol Haematol. December 2004 .
URL : http://AtlasGeneticsOncology.org/Tumors/SoftTisLeiomyoSarcID5122.html

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indexed on : Sat Feb 6 16:07:42 CET 2010

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