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Rhabdomyosarcoma: biology and therapy

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Diagnostic and Therapeutic Advances in Pediatric Oncology

Part of the book series: Cancer Treatment and Research ((CTAR,volume 92))

Abstract

Rhabdomyosarcoma, a malignant tumor of skeletal muscle, is the most common soft tissue sarcoma in people younger than 21 years, accounting for 5% to 8% of all cases of childhood cancer [1,2]. The traditional histologic classification scheme for rhabdomyosarcoma is primarily based on the degree of resemblance to normal fetal skeletal muscle prior to innervation [3]. These tumors are classified into two broad histiotypes, namely, embryonal and alveolar, that each have characteristic pathologic, cytogenetic, and clinical features [4]. Embryonal rhabdomyosarcomas occur in young children and account for approximately 60% of the cases; the primary tumor is usually located in specific anatomic sites, including the head and neck region, genitourinary tract, and orbit. The tumor is characterized by histologically variable numbers of malignant spindle and primitive round cells that may contain the cross-striations typical of skeletal muscle. By contrast, alveolar rhabdomyosarcomas often occur during adolescence as primary tumors of the extremities or trunk. This histologic variant is characterized by the presence of fibrovascular septa that form alveolar-like spaces filled with primitive, poorly cohesive, monomorphous, malignant cells. Patients with tumors of alveolar histology are generally considered to have a poorer clinical prognosis than do those with embryonal rhabdomyosarcomas [5].

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Authors
  1. Alberto S. Pappo
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  2. David N. Shapiro
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  1. Robert H. Lurie Cancer Center, Northwestern University Medical School, Chicago, Illinois, USA

    David O. Walterhouse M.D.  & Susan L. Cohn M.D.  & 

  2. Department of Pediatrics, Children’s Memorial Hospital, Chicago, Illinois, USA

    David O. Walterhouse M.D.  & Susan L. Cohn M.D.  & 

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Pappo, A.S., Shapiro, D.N. (1997). Rhabdomyosarcoma: biology and therapy. In: Walterhouse, D.O., Cohn, S.L. (eds) Diagnostic and Therapeutic Advances in Pediatric Oncology. Cancer Treatment and Research, vol 92. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-5767-8_10

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