Molecular pathology of sarcomas.

  title={Molecular pathology of sarcomas.},
  author={Daniel Osuna and Enrique de {\'A}lava},
  journal={Reviews on recent clinical trials},
  volume={4 1},
Bone and soft tissue sarcomas are an infrequent group of tumours with a prevalence of 4 in 100,000 people/year. Sarcomas, such as synovial sarcoma, Ewing's sarcoma and osteosarcoma, are more usual in adolescents or in young adults. Neoplasias such as leiomyosarcoma or liposarcoma are more frequent in patients over 55 years. One relevant topic is related to sarcomagenesis elucidation, a key for discovering the early molecular mechanisms involved in the development of sarcomas as well as the… 

Tables from this paper

The Clinical Relevance of Molecular Genetics in Soft Tissue Sarcomas

The discovery of the early mechanisms involved in the genesis of sarcomas, the more relevant signaling pathways, and the development of genetically engineered mouse models could also provide a new individualized therapeutic strategy against these tumors.

Molecular pathology of sarcomas: concepts and clinical implications

The molecular genetic changes that have been described in sarcomas over the past era have aided the understanding of their pathogenesis and can assist the pathologist in the differential diagnosis of some of these entities, especially within the groups of small blue round cell tumors and spindle cell tumors, if performed in specialized centers.

Molecular Analysis of a Recurrent Sarcoma Identifies a Mutation in FAF1

The results suggest that the tumor represents a recurrence of a dormant metastasis from an originally misdiagnosed neoplasm, and a loss of FAF1 function could cause constitutive WNT pathway activity.

The role of molecular pathology in mediastinal sarcomas

The role of molecular pathology is covered, specifically with regards to diagnosis, as well as discuss the salient molecular genetic features of the various types of sarcoma that occur within the mediastinum.

The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy

Preliminary clinical trials, supported by solid basic research and strong preclinical evidence, promises a new era in the clinical management of these broad spectrum of malignant tumors.

Managing sarcoma: where have we come from and where are we going?

There is a need for novel subtype-specific treatment strategies for metastatic and unresectable sarcoma subtypes with targeted therapies as well as promising new therapies that are currently underway in clinical trials.

New drugs in sarcomas

The Phase III studies with pazopanib, regorafenib, muramyl tripeptide (MTP) and ridaforolimus are extensively discussed as well as the biological rationale for the use of these compounds.

Novel Therapeutic Targets in Soft Tissue Sarcomas

The benefit of systemic therapy in the recurrent and/or metastatic setting, including doxorubicin, ifsofamide and DTIC, has been shown to be modest with an overall response rate of 10-15%, as single agent, and of 30% in combination at the expense of toxicity without any improvement to the median overall survival rate of 12 months.

Xenograft and genetically engineered mouse model systems of osteosarcoma and Ewing's sarcoma: tumor models for cancer drug discovery

The use of mouse sarcoma models for understanding the mechanisms involved in the response of tumors to new treatments is an important step in the process of drug discovery and the development of clinically relevant therapeutic strategies for these diseases.

Identification and validation of therapeutic targets in sarcoma models

SSX is identified and validated as an ideal molecular target for drug development in anti-cancer research and shown that SSX regulates cell cycle progression and is involved in tumor formation through its ability to activateβ-catenin signalling.



Soft tissue sarcomas of adults: state of the translational science.

  • E. BordenL. Baker M. Brennan
  • Medicine, Biology
    Clinical cancer research : an official journal of the American Association for Cancer Research
  • 2003
Sarcomas--like leukemias, which are also mesodermal malignancies--carry biological significance disproportionate to their clinical frequency. Identification of mutations and translocations associated

Molecular prognostication for soft tissue sarcomas: are we ready yet?

The recent debate around the prognostic value of synovial sarcoma fusion genes is an example of many of the issues encountered in studies of molecular prognostic markers in cancer, and what valuable lessons can be taken from these studies.

Chromosomal translocations and sarcomas

Basic scientific findings are reviewed that elucidate the aberrant functions of SYT-SSX in chromatin remodeling and of EWS-FLI1 in transcription and mRNA splicing that will help identify new therapeutic targets.

SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma.

The type of SYT-SSX fusion transcript correlates with both the histologic subtype and the clinical behavior of synovial sarcoma and may also yield important independent prognostic information.

Focus on sarcomas.

Development of Ewing's sarcoma from primary bone marrow-derived mesenchymal progenitor cells.

It is shown that EWS-FLI-1 alone can transform primary bone marrow-derived mesenchymal progenitor cells and generate tumors that display hallmarks of Ewing's sarcoma, including a small round cell phenotype, expression of EFT-associated markers, insulin like growth factor-I dependence, and induction or repression of numerous E WS-FLi-1 target genes.

Gene expression profiling of human sarcomas: insights into sarcoma biology.

An online, publicly available, searchable database housing the data from the gene expression profiles of these tumors is created, allowing the user to interactively explore this data set in depth.

Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family.

  • E. de ÁlavaW. Gerald
  • Biology, Medicine
    Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • 2000
The recent advances in the understanding of the molecular biology of ES and PNET represent a paradigm for the application of the basic biology of neoplasia to clinical management of patients.

Genome-wide analysis of gene expression in synovial sarcomas using a cDNA microarray.

Examination of genome-wide gene expression profiles of synovial sarcoma cases by cDNA microarray identified a set of genes that divided SS cases into two putative subclasses, a feature that may shed light on novel biological aspects of SS in addition to those having to do with epithelial differentiation.