Extremity rhabdomyosarcoma: Biological principles, staging, and treatment Journal Article


Author: LaQuaglia, M. P.
Article Title: Extremity rhabdomyosarcoma: Biological principles, staging, and treatment
Abstract: Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and adolescence with 20% of the primary tumors anatomically located on extremities. It is a complicated entity that requires careful planning and coordination between the surgical oncologist and other members of the therapeutic team for successful treatment. Even with the most effective regimens more than 50% of patients will die from progressive, usually distant disease. Further progress may require new therapeutic agents or techniques. The surgical oncologist is a necessary and often prominent member of the team. Elements of the biological behavior, histopathology, clinical staging, and treatment of extremity rhabdomyosarcomas occurring in children are discussed. In particular, the importance of the alveolar subtype in determination of prognostic risk as well as new findings regarding the molecular biologic determinants of phenotypic behavior are mentioned. Finally, innovative methods of local control like regional arterial perfusion and rapid intraoperative brachytherapy are addressed. Copyright © 1993 Wiley‐Liss, Inc., A Wiley Company
Keywords: cancer survival; child; cancer surgery; histopathology; review; cisplatin; doxorubicin; combined modality therapy; cancer staging; nuclear magnetic resonance imaging; lymph node metastasis; antineoplastic agent; neoplasm staging; genes; etoposide; cyclophosphamide; melphalan; vincristine; ifosfamide; limb; dactinomycin; extremities; physical examination; local control; molecular biology; rhabdomyosarcoma; soft tissue neoplasms; rhabdomyosarcoma, alveolar; myod protein; artery perfusion; prognosis; human; priority journal; alveolar subtype; arterial perfusion
Journal Title: Seminars in Surgical Oncology
Volume: 9
Issue: 6
ISSN: 8756-0437
Publisher: John Wiley & Sons  
Date Published: 1993-11-01
Start Page: 510
End Page: 519
Language: English
DOI: 10.1002/ssu.2980090609
PUBMED: 8284570
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 1 March 2019 -- Source: Scopus
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