Intergroup Rhabdomyosarcoma Study: Update for Pathologists

SAGE Publications - Tập 1 - Trang 550-561 - 1998
Stephen J. Qualman1, Cheryl M. Coffin2, William A. Newton1, Hiroshi Hojo3, Timothy J. Triche4, David M. Parham5, William M. Crist6
1Department 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
2Department 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
3Department of Pathology, Fukushima Medical College, 1 Hikariga-Oka, Fukushima 960-12, Japan , , JP
4Department 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
5Department of Pathology, Arkansas Children's Hospital and University of Arkansas Medical School, 800 Marshall Street, Slot 820, Little Rock, AR 72202-3591, USA , , US
6Department of Pediatric and Adolescent Medicine, Mayo Clinic, Desk East 9 (Peds), 200 First Street, SW, Rochester, MN 55905-0001, USA , , US

Tóm tắt

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.

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SAGE Publications

Tập 1

550-561

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