Pediatric and Developmental Pathology

, Volume 1, Issue 6, pp 550–561

Intergroup Rhabdomyosarcoma Study: Update for Pathologists

  • Stephen J.  Qualman
  • Cheryl M.  Coffin
  • William A.  Newton
  • Hiroshi  Hojo
  • Timothy J.  Triche
  • David M.  Parham
  • William M.  Crist

DOI: 10.1007/s100249900076

Cite this article as:
Qualman, S., Coffin, C., Newton, W. et al. Pediatr. Dev. Pathol. (1998) 1: 550. doi:10.1007/s100249900076

ABSTRACT

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, and 75% of such cases in the United States are reviewed at the Pathology Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG). The first four generations of IRSG therapeutic trials (IRS I–IV) and supportive pathologic studies have generated a new International Classification of Rhabdomyosarcoma (ICR) that offers new morphologic concepts to the practicing pathologist. The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies. Emerging ICR variants of RMS place the patient in widely divergent prognostic categories (superior, botryoid or spindle cell variants; poor, solid alveolar or diffusely anaplastic variants). The cardinal histopathologic features of the ICR combined with results of studies of fusion genes seen with t(1;13) and t(2;13) will help delineate therapeutic subgroups of RMS for the fifth generation (IRS V) of IRSG studies. Consequently, it is imperative for the practicing pathologist to be familiar with the practical workup and diagnosis of RMS in childhood.

Key words: rhabdomyosarcoma, embryonal, botryoid, spindle cell, alveolar, anaplasia

Copyright information

© Springer-Verlag New York Inc. 1998

Authors and Affiliations

  • Stephen J.  Qualman
    • 1
  • Cheryl M.  Coffin
    • 2
  • William A.  Newton
    • 1
  • Hiroshi  Hojo
    • 3
  • Timothy J.  Triche
    • 4
  • David M.  Parham
    • 5
  • William M.  Crist
    • 6
  1. 1.Department of Laboratory Medicine and IRSG Pathology Center, Children's Hospital and Ohio State University School of Medicine, 700 Children's Drive, Columbus, OH 43205, USA US
  2. 2.Department of Pathology, Division of Pediatric Pathology, University of Utah School of Medicine and Primary Children's Medical Center, 100 North Medical Drive, Salt Lake City, UT 84113, USA US
  3. 3.Department of Pathology, Fukushima Medical College, 1 Hikariga-Oka, Fukushima 960-12, Japan JP
  4. 4.Department of Pathology, Children's Hospital Los Angeles and University of Southern California School of Medicine, 4650 Sunset Boulevard, M543, Los Angeles, CA 90027, USA US
  5. 5.Department of Pathology, Arkansas Children's Hospital and University of Arkansas Medical School, 800 Marshall Street, Slot 820, Little Rock, AR 72202-3591, USA US
  6. 6.Department of Pediatric and Adolescent Medicine, Mayo Clinic, Desk East 9 (Peds), 200 First Street, SW, Rochester, MN 55905-0001, USA US