Desmoplastic small round cell tumor: from state of the art to future clinical prospects

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

doi:10.1080/14737140.2023.2200171

Desmoplastic small round cell tumor: from state of the art to future clinical prospects

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^*

^*Corresponding author for this work

Cancer and Genomic Sciences

Research output: Contribution to journal › Review article › peer-review

77 Downloads (Pure)

Abstract

Introduction: 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 overall survival between 17 and 60 months, with the 5-year overall survival rate in the range of 10–20%.

Area covered: This review discusses the treatment strategies used for DSRCT over the years, the state of the art of current treatments, and future clinical prospects.

Expert opinion: The unsatisfactory outcomes for patients with DSRCT warrant investigations into innovative treatment combinations. An international multidisciplinary and multi-stakeholder collaboration, involving both pediatric and adult sarcoma communities, is needed to propel preclinical model generation and drug development, and innovative clinical trial designs to enable the timely testing of treatments involving novel agents guided by biology to boost the chances of survival for patients with this devastating disease.

Original language	English
Pages (from-to)	471-484
Number of pages	14
Journal	Expert Review of Anticancer Therapy
Volume	23
Issue number	5
DOIs	https://doi.org/10.1080/14737140.2023.2200171
Publication status	Published - 10 Apr 2023

Bibliographical note

Funding Information:
This paper was not funded.

Publisher Copyright:
© 2023 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.

Keywords

biomarker
chemotherapy
Desmoplastic small round cell tumor
hyperthermic intraperitoneal chemotherapy
novel agents
prognosis
radiotherapy
surgery

ASJC Scopus subject areas

Oncology
Pharmacology (medical)

Access to Document

10.1080/14737140.2023.2200171Licence: Creative Commons: Attribution-NonCommercial-NoDerivs (CC BY-NC-ND)

HovsepyanS2023DesmoplasticFinal published version, 790 KBLicence: Creative Commons: Attribution-NonCommercial-NoDerivs (CC BY-NC-ND)

Cite this

Hovsepyan, S., Giani, C., Pasquali, S., Di Giannatale, A., Chiaravalli, S., Colombo, C., Orbach, D., Bergamaschi, L., Vennarini, S., Gatz, S. A., Gasparini, P., Berlanga, P., Casanova, M., & Ferrari, A. (2023). Desmoplastic small round cell tumor: from state of the art to future clinical prospects. Expert Review of Anticancer Therapy, 23(5), 471-484. https://doi.org/10.1080/14737140.2023.2200171

@article{6efcea3555414c6bad3c5d006109d083,

title = "Desmoplastic small round cell tumor: from state of the art to future clinical prospects",

abstract = "Introduction: 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 overall survival between 17 and 60 months, with the 5-year overall survival rate in the range of 10–20%. Area covered: This review discusses the treatment strategies used for DSRCT over the years, the state of the art of current treatments, and future clinical prospects. Expert opinion: The unsatisfactory outcomes for patients with DSRCT warrant investigations into innovative treatment combinations. An international multidisciplinary and multi-stakeholder collaboration, involving both pediatric and adult sarcoma communities, is needed to propel preclinical model generation and drug development, and innovative clinical trial designs to enable the timely testing of treatments involving novel agents guided by biology to boost the chances of survival for patients with this devastating disease.",

keywords = "biomarker, chemotherapy, Desmoplastic small round cell tumor, hyperthermic intraperitoneal chemotherapy, novel agents, prognosis, radiotherapy, surgery",

author = "Shushan Hovsepyan and Claudia Giani and Sandro Pasquali and {Di Giannatale}, Angela and Stefano Chiaravalli and Chiara Colombo and Daniel Orbach and Luca Bergamaschi and Sabina Vennarini and Gatz, {Susanne Andrea} and Patrizia Gasparini and Pablo Berlanga and Michela Casanova and Andrea Ferrari",

note = "Funding Information: This paper was not funded. Publisher Copyright: {\textcopyright} 2023 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.",

year = "2023",

month = apr,

day = "10",

doi = "10.1080/14737140.2023.2200171",

language = "English",

volume = "23",

pages = "471--484",

journal = "Expert Review of Anticancer Therapy",

issn = "1473-7140",

publisher = "Taylor & Francis",

number = "5",

}

Hovsepyan, S, Giani, C, Pasquali, S, Di Giannatale, A, Chiaravalli, S, Colombo, C, Orbach, D, Bergamaschi, L, Vennarini, S, Gatz, SA, Gasparini, P, Berlanga, P, Casanova, M & Ferrari, A 2023, 'Desmoplastic small round cell tumor: from state of the art to future clinical prospects', Expert Review of Anticancer Therapy, vol. 23, no. 5, pp. 471-484. https://doi.org/10.1080/14737140.2023.2200171

TY - JOUR

T1 - Desmoplastic small round cell tumor

T2 - from state of the art to future clinical prospects

AU - Hovsepyan, Shushan

AU - Giani, Claudia

AU - Pasquali, Sandro

AU - Di Giannatale, Angela

AU - Chiaravalli, Stefano

AU - Colombo, Chiara

AU - Orbach, Daniel

AU - Bergamaschi, Luca

AU - Vennarini, Sabina

AU - Gatz, Susanne Andrea

AU - Gasparini, Patrizia

AU - Berlanga, Pablo

AU - Casanova, Michela

AU - Ferrari, Andrea

PY - 2023/4/10

Y1 - 2023/4/10

N2 - Introduction: 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 overall survival between 17 and 60 months, with the 5-year overall survival rate in the range of 10–20%. Area covered: This review discusses the treatment strategies used for DSRCT over the years, the state of the art of current treatments, and future clinical prospects. Expert opinion: The unsatisfactory outcomes for patients with DSRCT warrant investigations into innovative treatment combinations. An international multidisciplinary and multi-stakeholder collaboration, involving both pediatric and adult sarcoma communities, is needed to propel preclinical model generation and drug development, and innovative clinical trial designs to enable the timely testing of treatments involving novel agents guided by biology to boost the chances of survival for patients with this devastating disease.

AB - Introduction: 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 overall survival between 17 and 60 months, with the 5-year overall survival rate in the range of 10–20%. Area covered: This review discusses the treatment strategies used for DSRCT over the years, the state of the art of current treatments, and future clinical prospects. Expert opinion: The unsatisfactory outcomes for patients with DSRCT warrant investigations into innovative treatment combinations. An international multidisciplinary and multi-stakeholder collaboration, involving both pediatric and adult sarcoma communities, is needed to propel preclinical model generation and drug development, and innovative clinical trial designs to enable the timely testing of treatments involving novel agents guided by biology to boost the chances of survival for patients with this devastating disease.

KW - biomarker

KW - chemotherapy

KW - Desmoplastic small round cell tumor

KW - hyperthermic intraperitoneal chemotherapy

KW - novel agents

KW - prognosis

KW - radiotherapy

KW - surgery

UR - http://www.scopus.com/inward/record.url?scp=85152434124&partnerID=8YFLogxK

U2 - 10.1080/14737140.2023.2200171

DO - 10.1080/14737140.2023.2200171

M3 - Review article

C2 - 37017324

AN - SCOPUS:85152434124

SN - 1473-7140

VL - 23

SP - 471

EP - 484

JO - Expert Review of Anticancer Therapy

JF - Expert Review of Anticancer Therapy

IS - 5

ER -

Desmoplastic small round cell tumor: from state of the art to future clinical prospects

Abstract

Bibliographical note

Keywords

ASJC Scopus subject areas

Access to Document

Fingerprint

Cite this