Relapsed Rhabdomyosarcoma
Relapsed rhabdomyosarcoma (RMS) represents a significant therapeutic challenge. Nearly one-third of patients diagnosed with localized RMS and over two-thirds of patients with metastatic RMS will experience disease recurrence following primary treatment, generally within three years. Clinical feature...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-02-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/4/804 |
| _version_ | 1797396255812354048 |
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| author | Christine M. Heske Leo Mascarenhas |
| author_facet | Christine M. Heske Leo Mascarenhas |
| author_sort | Christine M. Heske |
| collection | DOAJ |
| description | Relapsed rhabdomyosarcoma (RMS) represents a significant therapeutic challenge. Nearly one-third of patients diagnosed with localized RMS and over two-thirds of patients with metastatic RMS will experience disease recurrence following primary treatment, generally within three years. Clinical features at diagnosis, including primary site, tumor invasiveness, size, stage, and histology impact likelihood of relapse and prognosis post-relapse. Aspects of initial treatment, including extent of surgical resection, use of radiotherapy, and chemotherapy regimen, are also associated with post-relapse outcomes, as are features of the relapse itself, including time to relapse and extent of disease involvement. Although there is no standard treatment for patients with relapsed RMS, several general principles, including tissue biopsy confirmation of diagnosis, assessment of post-relapse prognosis, determination of the feasibility of additional local control measures, and discussion of patient goals, should all be part of the approach to care. Patients with features suggestive of a favorable prognosis, which include those with botryoid RMS or stage 1 or group I embryonal RMS (ERMS) who have had no prior treatment with cyclophosphamide, have the highest chance of achieving long-term cure when treated with a multiagent chemotherapy regimen at relapse. Unfortunately, patients who do not meet these criteria represent the majority and have poor outcomes when treated with such regimens. For this group, strong consideration should be given for enrollment on a clinical trial. |
| first_indexed | 2024-03-09T00:48:44Z |
| format | Article |
| id | doaj.art-8a7abb1d575c4892b12f27f0e7565088 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T00:48:44Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-8a7abb1d575c4892b12f27f0e75650882023-12-11T17:21:12ZengMDPI AGJournal of Clinical Medicine2077-03832021-02-0110480410.3390/jcm10040804Relapsed RhabdomyosarcomaChristine M. Heske0Leo Mascarenhas1Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USACancer and Blood Disease Institute, Children’s Hospital Los Angeles, Division of Hematology/Oncology, Department of Pediatrics and Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USARelapsed rhabdomyosarcoma (RMS) represents a significant therapeutic challenge. Nearly one-third of patients diagnosed with localized RMS and over two-thirds of patients with metastatic RMS will experience disease recurrence following primary treatment, generally within three years. Clinical features at diagnosis, including primary site, tumor invasiveness, size, stage, and histology impact likelihood of relapse and prognosis post-relapse. Aspects of initial treatment, including extent of surgical resection, use of radiotherapy, and chemotherapy regimen, are also associated with post-relapse outcomes, as are features of the relapse itself, including time to relapse and extent of disease involvement. Although there is no standard treatment for patients with relapsed RMS, several general principles, including tissue biopsy confirmation of diagnosis, assessment of post-relapse prognosis, determination of the feasibility of additional local control measures, and discussion of patient goals, should all be part of the approach to care. Patients with features suggestive of a favorable prognosis, which include those with botryoid RMS or stage 1 or group I embryonal RMS (ERMS) who have had no prior treatment with cyclophosphamide, have the highest chance of achieving long-term cure when treated with a multiagent chemotherapy regimen at relapse. Unfortunately, patients who do not meet these criteria represent the majority and have poor outcomes when treated with such regimens. For this group, strong consideration should be given for enrollment on a clinical trial.https://www.mdpi.com/2077-0383/10/4/804rhabdomyosarcomarelapserisk-factorsprognosischemotherapyclinical trials |
| spellingShingle | Christine M. Heske Leo Mascarenhas Relapsed Rhabdomyosarcoma Journal of Clinical Medicine rhabdomyosarcoma relapse risk-factors prognosis chemotherapy clinical trials |
| title | Relapsed Rhabdomyosarcoma |
| title_full | Relapsed Rhabdomyosarcoma |
| title_fullStr | Relapsed Rhabdomyosarcoma |
| title_full_unstemmed | Relapsed Rhabdomyosarcoma |
| title_short | Relapsed Rhabdomyosarcoma |
| title_sort | relapsed rhabdomyosarcoma |
| topic | rhabdomyosarcoma relapse risk-factors prognosis chemotherapy clinical trials |
| url | https://www.mdpi.com/2077-0383/10/4/804 |
| work_keys_str_mv | AT christinemheske relapsedrhabdomyosarcoma AT leomascarenhas relapsedrhabdomyosarcoma |