Soft Tissues: Soft Tissue Leiomyosarcoma

Identity

ICD-Topo	C470-C476,C478-C479,C490-C496,C498-C499 CONNECTIVE & SOFT TISSUE
ICD-Morpho	8890/3 Leiomyosarcoma, NOS
Atlas_Id	5122
Phylum	Soft Tissues::Leiomyosarcoma
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

Histopathological grading of soft tissue tumours. Prognostic significance in a prospective study of 278 consecutive cases.

Jensen OM, H&oring;gh J, Ostgaard SE, Nordentoft AM, Sneppen O

The Journal of pathology. 1991 ; 163 (1) : 19-24.

PMID 2002420

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

Human pathology. 2003 ; 34 (6) : 549-558.

PMID 12827608

Citation

This paper should be referenced as such :

Luo, JH

Soft tissue tumors: Soft tissue leiomyosarcoma

Atlas Genet Cytogenet Oncol Haematol. 2005;9(1):54-55.

Free journal version : [ pdf ]   [ DOI ]

On line version : http://AtlasGeneticsOncology.org/Tumors/SoftTisLeiomyoSarcID5122.html

Other genes implicated (Data extracted from papers in the Atlas) [ 11 ]

Genes	BCL6	ADGRE5	DBN1	EDIL3	HGF	KCNH1	LATS1	MCAM	MME	PF4V1
	SRSF3

External links

arrayMap	Topo ( C47,C49) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
Disease database	Soft Tissues: Soft Tissue Leiomyosarcoma

REVIEW articles	automatic search in PubMed
Last year articles	automatic search in PubMed

© Atlas of Genetics and Cytogenetics in Oncology and Haematology

indexed on : Fri Jun 30 11:25:11 CEST 2017

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