Abstract
Data about the prognostic factors of soft-tissue leiomyosarcomas and their correlation with molecular profile are limited. From 1990 to 2010, 586 adult patients with a primary soft-tissue leiomyosarcoma were included in the French Sarcoma Group (GSF) database after surgery of the primary tumor. Multivariate analyses were conducted by Cox regression model in a backward stepwise procedure. Genetic profiling was conducted for 73 cases. Median age was 59 years (range, 21-98 years). The median follow-up of patients alive was 46 months. The 5-year metastasis-free survival (MFS) rate was 51% (95% location and grade > I were independent adverse prognostic factors for MFS). The 5-year overall survival (OS) rate was 63% [95% confidence interval (CI), 59-67]. On multivariate analysis, age ≥ 60 years old, tumor size > 5 cm, deep location, and grade > I were independent adverse prognostic factors for OS. Molecular profiling identified specific clusters with activation of different biologic pathways: retroperitoneal leiomyosarcomas are characterized by overexpression of genes involved in muscle differentiation and nonretroperitoneal leiomyosarcomas characterized by overexpression of genes mainly involved in extracellular matrix, wounding, and adhesion pathways. The CINSARC signature but not comparative genomic hybridization (CGH) profiling was predictive of outcome. Soft-tissue leiomyosarcomas represent a heterogeneous group of tumors with at least two categories, retroperitoneal and extremities leiomyosarcomas, having specific clinical outcome and molecular features. Future clinical trials should consider this heterogeneity for a better stratification of patients.
Highlights
Molecular profiling identified specific clusters with activation of different biologic pathways: retroperitoneal leiomyosarcomas are characterized by overexpression of genes involved in muscle differentiation and nonretroperitoneal leiomyosarcomas characterized by overexpression of genes mainly involved in extracellular matrix, wounding, and adhesion pathways
The CINSARC signature but not comparative genomic hybridization (CGH) profiling was predictive of outcome
Soft-tissue leiomyosarcomas represent a heterogeneous group of tumors with at least two categories, retroperitoneal and extremities leiomyosarcomas, having specific clinical outcome and molecular features
Summary
These tumors occur mainly in adults in any location of the body (soft-tissue or viscera). Data related to the clinical outcome of soft-tissue leiomyosarcomas are mainly limited to small, single-institution, nonexhaustive, or out-of-date series [2,3,4,5,6,7]. Only few data about the molecular characteristics of leiomyosarcomas are available Most of such studies analyzed a small number of cases and/or mixed visceral and soft-tissue and primary and metastatic specimens [8,9,10,11,12,13,14,15,16,17,18]. The main objective of our study was to investigate the clinical outcome, the prognostic factors of soft-tissue leiomyosarcomas and the correlation between molecular profiles and clinical characteristics
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