scholarly journals Genetic Characterization, Current Model Systems and Prognostic Stratification in PAX Fusion-Negative vs. PAX Fusion-Positive Rhabdomyosarcoma

Genes ◽  
2021 ◽  
Vol 12 (10) ◽  
pp. 1500
Author(s):  
Carina A. Dehner ◽  
Amy E. Armstrong ◽  
Marielle Yohe ◽  
Jack F. Shern ◽  
Angela C. Hirbe
Keyword(s):  
Soft Tissue ◽  
Risk Stratification ◽  
Genetic Characterization ◽  
Current Model ◽  
Soft Tissue Sarcomas ◽  
Model Systems ◽  
Genetic Pathways ◽  
Genetic Abnormalities ◽  
Prognostic Stratification ◽  
Molecular Landscape

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents and accounts for approximately 2% of soft tissue sarcomas in adults. It is subcategorized into distinct subtypes based on histological features and fusion status (PAX-FOXO1/VGLL2/NCOA2). Despite advances in our understanding of the pathobiological and molecular landscape of RMS, the prognosis of these tumors has not significantly improved in recent years. Developing a better understanding of genetic abnormalities and risk stratification beyond the fusion status are crucial to developing better therapeutic strategies. Herein, we aim to highlight the genetic pathways/abnormalities involved, specifically in fusion-negative RMS, assess the currently available model systems to study RMS pathogenesis, and discuss available prognostic factors as well as their importance for risk stratification to achieve optimal therapeutic management.

Genetic abnormalities in soft tissue sarcomas – implications for treatment

2006 ◽  
Vol 17 (6) ◽  
pp. 551-557 ◽  
Author(s):  
R Lor Randall ◽  
David M Cearley ◽  
Brian Johnson ◽  
David E Joyner
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Genetic Abnormalities

Neoadjuvant chemotherapy in high‐risk soft tissue sarcomas: A Sarculator‐based risk stratification analysis of the ISG‐STS 1001 randomized trial

Cancer ◽  
2021 ◽  
Author(s):  
Sandro Pasquali ◽  
Emanuela Palmerini ◽  
Vittorio Quagliuolo ◽  
Javier Martin‐Broto ◽  
Antonio Lopez‐Pousa ◽  
...  
Keyword(s):  
High Risk ◽  
Soft Tissue ◽  
Neoadjuvant Chemotherapy ◽  
Risk Stratification ◽  
Randomized Trial ◽  
Soft Tissue Sarcomas

scholarly journals Soft Tissue Sarcoma: An Insight on Biomarkers at Molecular, Metabolic and Cellular Level

Cancers ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 3044
Author(s):  
Serena Pillozzi ◽  
Andrea Bernini ◽  
Ilaria Palchetti ◽  
Olivia Crociani ◽  
Lorenzo Antonuzzo ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Predictive Biomarkers ◽  
Cellular Level ◽  
Comprehensive Overview ◽  
Novel Treatments ◽  
Patients At Risk ◽  
Prognostic Stratification ◽  
Biomarker Research ◽  
Metabolic Biomarkers

Soft tissue sarcomas (STSs) are a heterogeneous group of rare tumors. Although constituting only 1% of all human malignancies, STSs represent the second most common type of solid tumors in children and adolescents and comprise an important group of secondary malignancies. Over 100 histologic subtypes have been characterized to date (occurring predominantly in the trunk, extremity, and retroperitoneum), and many more are being discovered due to molecular profiling. STS mortality remains high, despite adjuvant chemotherapy. New prognostic stratification markers are needed to help identify patients at risk of recurrence and possibly apply more intensive or novel treatments. Recent scientific advancements have enabled a more precise molecular characterization of sarcoma subtypes and revealed novel therapeutic targets and prognostic/predictive biomarkers. This review aims at providing a comprehensive overview of the most relevant cellular, molecular and metabolic biomarkers for STS, and highlight advances in STS-related biomarker research.

LOCAL RECURRENCES AFTER THE TREATMENT OF SOFT TISSUE MALIGNANT FIBROUS HISTIOCYTOMA (UNCLASSIFIEDPLEOMORPHIC SARCOMA) OF THE LIMBS

2019 ◽  
Vol 72 (8) ◽  
pp. 1523-1526
Author(s):  
Oleksandr O. Lytvynenko ◽  
Volodymyr F. Konovalenko ◽  
Anton Yu. Ryzhov
Keyword(s):  
Soft Tissue ◽  
Malignant Fibrous Histiocytoma ◽  
Fibrous Histiocytoma ◽  
Survival Rates ◽  
Soft Tissue Sarcomas ◽  
Primary Treatment ◽  
Radical Excision ◽  
Local Recurrences ◽  
Results Of Treatment

Introduction: The treatment of patients with malignant fibrous histiocytoma as well as other soft tissue sarcomas is not sufficiently effective up to date, and has largely changed and reflects the alterations, occurred in oncology as a whole. The number of amputation decreased over the last 10-15 years. Some researchers associate the improvement of treatment outcomes with the development of combined and complex methods. The aim of the study is an improvement of the results of treatment of patients with soft tissue malignant histiocytoma on the basis of determination of factors, influencing local recurrence development. Materials and methods: The basis of our study was a comprehensive analysis of examination and treatment results of 130 patients with MFH of the soft tissue of limbs, of them in 84 patients (64.6%) the recurrences developed. The group included 45 (53.6%) males and 39 (46.4%) females. The major part of patients – 82.1% (60 patients) were older than 40 years. Results and conclusions: The number of recurrences after the treatment in general surgical facilities is 86.9%, whereas in the patients after the treatment in the specialized oncological facilities this figure is twice lower (40%). The characteristic of the medical facility where the patient receives his/her primary treatment largely affects the development of local recurrences, patients’ quality of life and overall survival rates. The surgical method remains the leading modality in the treatment of MFH of ST. Wide and radical excision of tumors in the specialized oncological facilities allows achieving better survival outcomes of the patients.

Sign in / Sign up

Export Citation Format

Share Document