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Endoglin Expression and Microvessel Density as Prognostic Factors in Pediatric Rhabdomyosarcoma

Relapsed Rhabdomyosarcoma

Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
Cancer 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, USA
Author to whom correspondence should be addressed.
Academic Editors: Sanjeev A. Vasudeva and Wendy Allen-Rhoades
J. Clin. Med. 2021, 10(4), 804;
Received: 31 December 2020 / Revised: 2 February 2021 / Accepted: 13 February 2021 / Published: 17 February 2021
(This article belongs to the Special Issue Current Advances in Pediatric 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 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. View Full-Text
Keywords: rhabdomyosarcoma; relapse; risk-factors; prognosis; chemotherapy; clinical trials rhabdomyosarcoma; relapse; risk-factors; prognosis; chemotherapy; clinical trials
MDPI and ACS Style

Heske, C.M.; Mascarenhas, L. Relapsed Rhabdomyosarcoma. J. Clin. Med. 2021, 10, 804.

AMA Style

Heske CM, Mascarenhas L. Relapsed Rhabdomyosarcoma. Journal of Clinical Medicine. 2021; 10(4):804.

Chicago/Turabian Style

Heske, Christine M., and Leo Mascarenhas. 2021. "Relapsed Rhabdomyosarcoma" Journal of Clinical Medicine 10, no. 4: 804.

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