Desmoplastic small round cell tumor: from state of the art to future clinical prospects
Expert Rev Anticancer Ther. 2023 Apr 5. doi: 10.1080/14737140.2023.2200171. Online ahead of print.ABSTRACTINTRODUCTION: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive soft tissue sarcoma, presenting mainly in male adolescents and young adults with multiple nodules disseminated within the abdominopelvic cavity. Despite a multimodal approach including aggressive cytoreductive surgery, intensive multi-agent chemotherapy, and postoperative whole abdominopelvic radiotherapy, the prognosis for DSRCT remains dismal. Median progression-free survival ranges between 4 and 21 months, and overal...
Source: Expert Review of Anticancer Therapy - April 5, 2023 Category: Cancer & Oncology Authors: Shushan Hovsepyan Claudia Giani Sandro Pasquali Angela Di Giannatale Stefano Chiaravalli Chiara Colombo Daniel Orbach Luca Bergamaschi Sabina Vennarini Susanne Andrea Gatz Patrizia Gasparini Pablo Berlanga Michela Casanova Andrea Ferrari Source Type: research

Desmoplastic small round cell tumor: from state of the art to future clinical prospects
Expert Rev Anticancer Ther. 2023 Apr 10:1-14. doi: 10.1080/14737140.2023.2200171. Online ahead of print.ABSTRACTINTRODUCTION: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive soft tissue sarcoma, presenting mainly in male adolescents and young adults with multiple nodules disseminated within the abdominopelvic cavity. Despite a multimodal approach including aggressive cytoreductive surgery, intensive multi-agent chemotherapy, and postoperative whole abdominopelvic radiotherapy, the prognosis for DSRCT remains dismal. Median progression-free survival ranges between 4 and 21 months, and ...
Source: Expert Review of Anticancer Therapy - April 5, 2023 Category: Cancer & Oncology Authors: Shushan Hovsepyan Claudia Giani Sandro Pasquali Angela Di Giannatale Stefano Chiaravalli Chiara Colombo Daniel Orbach Luca Bergamaschi Sabina Vennarini Susanne Andrea Gatz Patrizia Gasparini Pablo Berlanga Michela Casanova Andrea Ferrari Source Type: research

Identification of immunotherapy and radioimmunotherapy targets on desmoplastic small round cell tumors
ConclusionsWe propose that these TAAs should be further investigated in preclinical models as targets for immunotherapy and radioimmunotherapy with the hope of providing a rationale to extend these therapies to patients with advanced DSRCT. (Source: Frontiers in Oncology)
Source: Frontiers in Oncology - April 5, 2023 Category: Cancer & Oncology Source Type: research

Long ‐term survivors with desmoplastic small round cell tumor (DSRCT): Results from a retrospective single‐institution case series analysis
ConclusionsIn our series cure was likely achieved in 13% of DSRCT. LTS had no liver/extra-abdominal disease, were treated with complete surgery, and possibly WAP-RT/MC. (Source: Cancer Medicine)
Source: Cancer Medicine - March 23, 2023 Category: Cancer & Oncology Authors: Claudia Giani, Stefano Radaelli, Rosalba Miceli, Lorenza Gandola, Claudia Sangalli, Anna Maria Frezza, Salvatore Provenzano, Sandro Pasquali, Rossella Bertulli, Marco Fiore, Dario Callegaro, Michela Casanova, Stefano Chiaravalli, Paola Collin Tags: RESEARCH ARTICLE Source Type: research

Cancers, Vol. 15, Pages 1623: Routine EWS Fusion Analysis in the Oncology Clinic to Identify Cancer-Specific Peptide Sequence Patterns That Span Breakpoints in Ewing Sarcoma and DSRCT
Conclusions and future perspectives: our method allows interpretation of what peptides result from the in-frame translation of EWS fusion junctions. These sequences, coupled with HLA-peptide binding data, are used to identify potential sequences of cancer-specific immunogenic peptides for Ewing sarcoma or DSRCT patients. This information may also be useful for immune monitoring (e.g., circulating T-cells with fusion-peptide specificity) to detect vaccine candidates, responses, or residual disease. (Source: Cancers)
Source: Cancers - March 6, 2023 Category: Cancer & Oncology Authors: Peter M. Anderson Zheng Jin Tu Scott E. Kilpatrick Matteo Trucco Rabi Hanna Timothy Chan Tags: Communication Source Type: research

Molecular profiling in desmoplastic small round cell tumours
Int J Biochem Cell Biol. 2023 Feb 1:106383. doi: 10.1016/j.biocel.2023.106383. Online ahead of print.ABSTRACTDesmoplastic small round cell tumour (DSRCT) is an ultra-rare soft tissue sarcoma that is characterised by aggressive disease and dismal patient outcomes. Despite multi-modal therapy, prognosis remains poor and there are currently no effective targeted therapies available for patients with this disease. Advances in comprehensive molecular profiling approaches including next generation sequencing and proteomics hold the promise of identifying new therapeutic targets and biomarkers. In this review, we provide an overv...
Source: The International Journal of Biochemistry and Cell Biology - February 3, 2023 Category: Biochemistry Authors: Yuen Bun Tam Robin L Jones Paul H Huang Source Type: research

Molecular profiling in desmoplastic small round cell tumours
Int J Biochem Cell Biol. 2023 Feb 1:106383. doi: 10.1016/j.biocel.2023.106383. Online ahead of print.ABSTRACTDesmoplastic small round cell tumour (DSRCT) is an ultra-rare soft tissue sarcoma that is characterised by aggressive disease and dismal patient outcomes. Despite multi-modal therapy, prognosis remains poor and there are currently no effective targeted therapies available for patients with this disease. Advances in comprehensive molecular profiling approaches including next generation sequencing and proteomics hold the promise of identifying new therapeutic targets and biomarkers. In this review, we provide an overv...
Source: The International Journal of Biochemistry and Cell Biology - February 3, 2023 Category: Biochemistry Authors: Yuen Bun Tam Robin L Jones Paul H Huang Source Type: research

Transcription regulators and ultra-rare and other rare translocation-related sarcomas treated with trabectedin: A proof of principle from a post-hoc analysis
ConclusionsTrabectedin has some anti-tumor activity in some ultra-rare and other rare sarcomas, particularly translocation-related sarcomas, with the well-known manageable safety profile. (Source: Frontiers in Oncology)
Source: Frontiers in Oncology - December 8, 2022 Category: Cancer & Oncology Source Type: research