Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treate...

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Autores principales:	Madelyn Espinosa-Cotton, Nai-Kong V. Cheung
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	DSRCT = desmoplastic small round cell tumor antibodies immunotherapy targeted therapy radioimmunotherapy CAR T cell Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/464a5e21d0bd42d384162eed6cb0f7ce
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spelling	oai:doaj.org-article:464a5e21d0bd42d384162eed6cb0f7ce2021-11-19T05:07:59ZImmunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor2234-943X10.3389/fonc.2021.772862https://doaj.org/article/464a5e21d0bd42d384162eed6cb0f7ce2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fonc.2021.772862/fullhttps://doaj.org/toc/2234-943XDesmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treated with a combination of aggressive chemotherapy, radiation, and surgery. Targeted therapy inhibitors of PDGFA and IGF-1R, which are almost uniformly overexpressed in DSRCT, have largely failed in clinical trials. As in cancer in general, interest in immunotherapy to treat DSRCT has increased in recent years. To that end, several types of immunotherapy are now being tested clinically, including monoclonal antibodies, radionuclide-conjugated antibodies, chimeric antigen receptor T cells, checkpoint inhibitors, and bispecific antibodies (BsAbs). These types of therapies may be particularly useful in DSRCT, which is frequently characterized by widespread intraperitoneal implants, which are difficult to completely remove surgically and are the frequent cause of relapse. Successful treatment with immunotherapy or radioimmunotherapy following debulking surgery could eradiate these micrometasteses and prevent relapse. Although there has been limited success to date for immunotherapy in pediatric solid tumors, the significant improvements in survival seen in the treatment of other pediatric solid tumors, such as metastatic neuroblastoma and its CNS spread, suggest a potential of immunotherapy and specifically compartmental immunotherapy in DSRCT.Madelyn Espinosa-CottonNai-Kong V. CheungFrontiers Media S.A.articleDSRCT = desmoplastic small round cell tumorantibodiesimmunotherapytargeted therapyradioimmunotherapyCAR T cellNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENFrontiers in Oncology, Vol 11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	DSRCT = desmoplastic small round cell tumor antibodies immunotherapy targeted therapy radioimmunotherapy CAR T cell Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	DSRCT = desmoplastic small round cell tumor antibodies immunotherapy targeted therapy radioimmunotherapy CAR T cell Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Madelyn Espinosa-Cotton Nai-Kong V. Cheung Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
description	Desmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treated with a combination of aggressive chemotherapy, radiation, and surgery. Targeted therapy inhibitors of PDGFA and IGF-1R, which are almost uniformly overexpressed in DSRCT, have largely failed in clinical trials. As in cancer in general, interest in immunotherapy to treat DSRCT has increased in recent years. To that end, several types of immunotherapy are now being tested clinically, including monoclonal antibodies, radionuclide-conjugated antibodies, chimeric antigen receptor T cells, checkpoint inhibitors, and bispecific antibodies (BsAbs). These types of therapies may be particularly useful in DSRCT, which is frequently characterized by widespread intraperitoneal implants, which are difficult to completely remove surgically and are the frequent cause of relapse. Successful treatment with immunotherapy or radioimmunotherapy following debulking surgery could eradiate these micrometasteses and prevent relapse. Although there has been limited success to date for immunotherapy in pediatric solid tumors, the significant improvements in survival seen in the treatment of other pediatric solid tumors, such as metastatic neuroblastoma and its CNS spread, suggest a potential of immunotherapy and specifically compartmental immunotherapy in DSRCT.
format	article
author	Madelyn Espinosa-Cotton Nai-Kong V. Cheung
author_facet	Madelyn Espinosa-Cotton Nai-Kong V. Cheung
author_sort	Madelyn Espinosa-Cotton
title	Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
title_short	Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
title_full	Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
title_fullStr	Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
title_full_unstemmed	Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor
title_sort	immunotherapy and radioimmunotherapy for desmoplastic small round cell tumor
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/464a5e21d0bd42d384162eed6cb0f7ce
work_keys_str_mv	AT madelynespinosacotton immunotherapyandradioimmunotherapyfordesmoplasticsmallroundcelltumor AT naikongvcheung immunotherapyandradioimmunotherapyfordesmoplasticsmallroundcelltumor
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Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor

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