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

@article{Quesada2012TheMB,
  title={The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy},
  author={Jorge R. Quesada and Robert J. Amato},
  journal={Sarcoma},
  year={2012},
  volume={2012}
}
Basic research in sarcoma models has been fundamental in the discovery of scientific milestones leading to a better understanding of the molecular biology of cancer. Yet, clinical research in sarcoma has lagged behind other cancers because of the multiple clinical and pathological entities that characterize sarcomas and their rarity. Sarcomas encompass a very heterogeneous group of tumors with diverse pathological and clinical overlapping characteristics. Molecular testing has been fundamental… 

Figures and Tables from this paper

The role of inflammation in sarcoma.

  • J. Radons
  • Biology, Medicine
    Advances in experimental medicine and biology
  • 2014
TLDR
This chapter gives an overview of actual therapeutic standards, newest evidence-based studies and exciting options for targeted therapies in sarcomas.

The EPH/Ephrin System in Bone and Soft Tissue Sarcomas’ Pathogenesis and Therapy: New Advancements and a Literature Review

TLDR
Based on published PubMed literature, significant interactions between the EPH/ephrin signaling pathway and a plethora of normal and abnormal cascades contribute to molecular mechanisms enhancing malignancy during sarcoma progression.

Molecular targeted therapies in non-GIST soft tissue sarcomas: what the radiologist needs to know

TLDR
The purpose of this article is to provide a primer for the radiologist on the various molecular targeted therapies in advanced/metastatic non-gastrointestinal stromal soft tissue sarcomas with emphasis on the role of imaging in assessing treatment response and complications.

Trabectedin for the treatment of soft tissue sarcomas

TLDR
Toxicity profile is acceptable and manageable with no reported cumulative toxicities, therefore, trabectedin has become one relevant therapeutic option in metastatic STS, especially in selected histologies.

Metastatic sarcoma: tailored strategies for a heterogeneous disease

TLDR
The state of the art and future perspectives for the management of soft tissue sarcomas are presented and the combination of systemic and local treatments can often be proposed.

The T-box transcription factor 3 is a promising biomarker and a key regulator of the oncogenic phenotype of a diverse range of sarcoma subtypes

TLDR
It is revealed that TBX3 may exhibit oncogene or tumour suppressor activity in sarcomas, which suggests that its role in cancer progression may rely on cellular context.

Proteomic approach toward personalized sarcoma treatment: Lessons from prognostic biomarker discovery in gastrointestinal stromal tumor

TLDR
Pfetin (potassium channel tetramerization domain containing 12, KCTD 12) is identified as a novel prognostic biomarker for sarcoma, and immunohistochemically confirmed its clinical usefulness by a multiinstitutional validation study.

Emerging therapies for adult soft tissue sarcoma

TLDR
Since peculiar chemosensitivity towards alternative drugs was described for different metastatic subtypes in second or further lines, the modern concept of ‘histology-driven chemotherapy’ has been accepted and employed: gemicitabine ± dacarbazine, trabectedin and taxanes used respectively in patients with leiomyosarcoma, solitary fibrous tumor, myxoid/round cell liposar coma, angiosarcomA.

Inflammatory stress and sarcomagenesis: a vicious interplay

  • J. Radons
  • Medicine, Biology
    Cell Stress and Chaperones
  • 2013
TLDR
An overview of preclinical and clinical evidence of the molecular pathways involved in the inflammatory stress response in sarcomagenesis and the most targeted therapies is made.
...

References

SHOWING 1-10 OF 113 REFERENCES

Molecular pathology of sarcomas: concepts and clinical implications

TLDR
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.

Recent advances in the molecular pathology of soft tissue sarcoma: Implications for diagnosis, patient prognosis, and molecular target therapy in the future

TLDR
In malignant rhabdoid tumor and epithelioid sarcoma, frequent alteration of the SMARCB1/INI1tumor‐suppressor gene and the loss of its protein have been demonstrated, indicating that this molecule could be an effective target of these sarcomas.

Molecular Approaches to Sarcoma Therapy

TLDR
This review summarizes published approaches targeting a specific molecular mechanism associated with sarcomagenesis and discusses the potential for improving outcomes in sarcoma patients and the conceptual obstacles to be overcome.

Molecular pathology of sarcomas.

TLDR
This review points out the clinical projection of sarcomagenesis elucidation and knowledge of diverse types of molecular alterations in sarcomas, as well as the identification of reliable molecular markers and possible therapeutic targets.

Genomic characteristics of soft tissue sarcomas

TLDR
The genomic characterization of soft tissue sarcomas has not only provided cell biologists with decisive information on the parts of the genome that may harbor genes that are essential for tumor development but also given the clinicians involved in the management of these patients a valuable diagnostic tool.

New Developments in Mammalian Target of Rapamycin Inhibitors for the Treatment of Sarcoma

TLDR
Evidence from both preclinical and clinical studies supports further study of mTOR‐targeting rapalogs in the treatment of various subtypes of sarcoma, and suggests that mTOR is a potentially important therapeutic target in these diseases.

Targeted Anti-Cancer Therapies for Renal Cancer

TLDR
On the basis of the limited studies to date, targeted therapies offer the greatest clinical benefit in the management of renal cell cancer, although additional basic research is still warranted to further improve clinical outcome.

The emerging role of mammalian target of rapamycin inhibitors in the treatment of sarcomas

TLDR
The PI3K–Akt–mTOR pathway is therefore an exciting target for therapy of sarcomas, and its blockade represents an opportunity to improve outcomes in this poor-prognosis disease.

The insulin‐like growth factor system and sarcomas

TLDR
Preclinical data suggest that the IGF system could be a promising target for therapy in these sarcomas, and the occasional occurrence of paraneoplastic hypoglycaemia as a result of the secretion of incompletely processed forms of pro‐IGF‐II by sarcoma is discussed.
...