Novel treatment strategies for soft tissue sarcoma (original) (raw)
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Curent concepts in pathology of soft tissue sarcoma
Indian journal of surgical oncology, 2011
Soft tissue sarcomas (STS) constitute a heterogeneous category of soft tissue neoplasia composed mostly of uncommon tumors of diverse histology, different biology and varied outcomes. Substantial developments in immunohistochemistry (IHC), cytogenetics and molecular genetics of STS have caused a significant change in the classification and diagnosis of these tumors with a direct implication for clinical management and prognosis. In this review we discuss newer developments impacting diagnosis and prediction.
Advances in Molecular Diagnosis and Personalized Treatment of Soft Tissue Sarcomas
INTERNATIONAL RESEARCH JOURNAL OF ONCOLOGY , 2024
Soft tissue tumors are a diverse range of diagnostic entities including the majority of them benign in nature and behavior. Soft tissue sarcomas are uncommon tumors that represent 1% of all malignancies and are classified as malignant entities. The wide variety of tumor types and pathological overlap across the tumor entities make the diagnosis of soft tissue tumors difficult for pathologists. With the advancement of molecular genetic tools, our understanding of the molecular pathogenesis of soft tissue tumors has been significantly important in diagnosis, treatment, and prognosis. The role of surgery, chemotherapy, radiotherapy, target therapy, and immunotherapy are needed to identify patients who benefit from each type of treatment. This review aims to provide an update on recently described diagnostic methods and management of soft tissue sarcomas.
Molecular classification of soft tissue sarcomas and its clinical applications
International journal of clinical and experimental pathology, 2010
Sarcomas are a heterogeneous group of tumors that are traditionally classified according to the morphology and type of tissue that they resemble, such as rhabdomyosarcoma, which resembles skeletal muscle. However, the cell of origin is unclear in numerous sarcomas. Molecular genetics analyses have not only assisted in understanding the molecular mechanism in sarcoma pathogenesis but also demonstrated new relationships within different types of sarcomas leading to a more proper classification of sarcomas. Molecular classification based on the genetic alteration divides sarcomas into two main categories: (i) sarcomas with specific genetic alterations; which can further be subclassified based on a) reciprocal translocations resulting in oncogenic fusion transcripts (e.g. EWSR1-FLI1 in Ewing sarcoma) and b) specific oncogenic mutations (e.g. KIT and PDGFRA mutations in gastrointestinal stromal tumors) and (ii) sarcomas displaying multiple, complex karyotypic abnormalities with no specif...
The Clinical Relevance of Molecular Genetics in Soft Tissue Sarcomas
Advances in Anatomic Pathology, 2010
Bone and soft tissue sarcomas are an infrequent and heterogeneous group of mesenchymal tumors including more than a hundred different entities attending to histologic patterns. Research into the molecular aspects of sarcomas has increased greatly in the last few years. This enormous amount of knowledge has allowed, for instance, to refine the classification of sarcomas, improve the diagnosis, and increase the number of therapeutical targets available, most of them under preclinical evaluation. However, other important key issues, such as sarcomagenesis and the cell of origin of sarcomas, remain unresolved. From a molecular point of view, these neoplasias are grouped into 2 main types: (a) sarcomas showing relatively simple karyotypes and translocations, which originate gene fusions (eg, EWS-FLI1 in Ewing sarcoma) or point mutations (eg, c-kit in the gastrointestinal tumors) and (b) sarcomas showing unspecific gene alterations, very complex karyotypes, and no translocations. 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. This review describes the clinical application of some of the molecular alterations found in sarcomas, some advances in the field of sarcomagenesis, and the development of animal models.
Indian Journal of Medical and Paediatric Oncology, 2022
Soft tissue sarcomas (STS) are a diverse group of rare solid tumors of mesenchymal cell origin with distinct clinical and pathological features. They account for less than 1% of all adult malignancies and 15% of pediatric neoplasms. They include over hundreds of different histological subtypes. Many of these subtypes can occur at any age and are not confined to a specific site. Each subtype displays variable clinical behavior. Low incidence, variable presentation, behavior, and long-term outcomes further make it challenging to treat. There are multiple ongoing trials that focus on the anatomic site and histologic subtype to tailor the treatment. Further rarity of each histotype is a major barrier to recruit patients to randomized controlled trials. A multidisciplinary approach is mandatory in all cases of soft tissue sarcomas.The purpose of this review is to thoroughly understand the existing literature on history, incidence, epidemiology, etiology, histology, pathogenesis, diagnost...
The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy
Sarcoma, 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 in the identification and better definition of more specific entities among this vast array of malignancies. A group of sarcomas are distinguished by specific molecular aberrations such as somatic mutations, intergene deletions, gene amplifications, reciprocal translocations, and complex karyotypes. These and other discoveries have led to a better understanding of the growth signals and the molecular pathways involved in the development of these tumors. These findings are leading to treatment strategies currently under intense investigation. Disruption of the growth signals is being targeted with antagonistic antibodies, tyrosine kinase inhibitors, and inhibitors of several downstream molecules in diverse molecular pathways. 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.
Advances in the Pathology and Molecular Biology of Sarcomas and the Impact on Treatment
Sarcomas are a complex group of childhood and adult neoplasms with differentiation towards mesenchymal tissues that can occur at almost every anatomic site. Although pathologically diverse, they frequently show similar clinical presentations and radiological findings, such that correct histopathologic diagnosis, utilising the appropriate ancillary immunohistochemical and molecular techniques, underpins their management. This article gives an overview of the pathology, coupled with recent advances in molecular biology, of a selection of soft tissue sarcomas from a clinicopathological perspective, discussing histopathological diagnosis with developments in molecular diagnosis and the incorporation of these findings into diagnostic practice and current and potential targeted treatments.
Soft Tissue Sarcomas: An Overview on Histomorphology
Annals of SBV
Background: Soft tissue sarcomas (STS) comprise a diverse group of rare malignancies that arise from connective tissues. The natural course of STS is unpredictable and aggressive if not diagnosed at an early stage. Discussion: The subclassification of these tumors is important for prognosis and clinical management of patients. In the present review, we discuss the histomorphologic features of STS and its subtypes based on 2013 World Health Organization classification and throw light on the incidence and presentation of STS. Conclusion: The systematic approach to morphologic assessment of STS outlined in this review will lead to narrowing of differential diagnoses and pave way for effective diagnosis and implementation of treatment strategies.