Rhabdomyosarcomas in adults and children: An update

Department of Pathology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock 72202, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.88). 11/2006; 130(10):1454-65. DOI: 10.1043/1543-2165(2006)130[1454:RIAACA]2.0.CO;2
Source: PubMed

ABSTRACT Rhabdomyosarcomas comprise a relatively common diagnostic entity among childhood cancers and a relatively rare one among adult tumors. They may possess a variety of histologies that generally differ among age groups. These lesions appear to be separate biologic entities as well as morphologic categories, with embryonal tumors having genetic lesions related to loss of heterozygosity and aberrant parental imprinting, alveolar tumors containing genetic fusions between PAX and forkhead genes, and pleomorphic tumors showing an accumulation of genetic lesions similar to other adult high-grade sarcomas.
To present guidelines for diagnosis of rhabdomyosarcoma and recent finding concerning the biology and classification of these lesions.
Review of recent and older published literature and distillation of the authors' experience.
Infants and young children tend to have embryonal rhabdomyosarcomas, adolescents and young adults tend to have alveolar rhabdomyosarcomas, and older adults tend to have pleomorphic rhabdomyosarcomas, although there is some overlap. Newer rare entities, including spindle cell rhabdomyosarcoma and sclerosing rhabdomyosarcoma, have been described in children and adults. Fusion-positive tumors have a distinct molecular signature with downstream activation of a number of myogenic and tumorigenic factors. Genetic testing may be successfully used for diagnosis and may guide therapy in future clinical trials. Differential diagnosis has become simpler than in previous years, because of use of myogenic factors in immunohistochemistry, but classification based solely on histologic features remains challenging.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: The treatment of rhabdomyosarcoma (RMS) remains challenging, with metastatic and alveolar RMS offering a particularly poor prognosis. Therefore, the identification and evaluation of novel antigens, which are suitable targets for immunotherapy, is one attractive possibility to improve the treatment of this disease. Here we show that chondroitin sulfate proteoglycan 4 (CSPG4) is expressed on RMS cell lines and RMS patient material. We evaluated the immunotoxin (IT) αMCSP-ETA', which specifically recognizes CSPG4 on the RMS cell lines RD, FL-OH1, TE-671 and Rh30. It is internalized rapidly, induces apoptosis and thus kills RMS cells selectively. We also demonstrate the specific binding of this IT to RMS primary tumor material from three different patients.
    Cancer Letters 07/2014; 352(2). DOI:10.1016/j.canlet.2014.07.006 · 5.02 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background. Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. Fifty percent of RMS cases occur in the first 10 years of life and less commonly in infants younger than one-year old. These infants require adapted multimodality treatment approaches. Patients and Methods. We analyzed patients' characteristics, treatment modalities, and the outcome for RMS infants treated at Children's Cancer Hospital Egypt (CCHE) between July 2007 and December 2010 and compared them to patients above one year treated on the same protocol. Results. Out of the 126 RMS treated during this period, 18 were below the age of one year. The male: female ratio was 1.25 : 1. The median age at diagnosis was 0.7 ± 0.2 years. Most of the cases (27.8%) were presented in head and neck regions. The estimated 4-years failure-free survival and overall survival for infants were 49 ± 12% and 70 ± 12%, respectively. These failure-free survival rate and overall survival rate did not differ from those for older patients (P = 0.2). Conclusion. Infants with RMS are a unique group of RMS who needs special concerns in tailoring treatment in addition to concerns regarding toxicity and morbidity in infants.
    Sarcoma 01/2013; 2013:439213. DOI:10.1155/2013/439213
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Introduction. The most common sarcoma of childhood is rhabdomyosarcoma, approximately 35% of all paediatric rhabdomyosarcomas occur in the head and neck. Case Report. A 3-year-old girl referred to our clinic due to serosanguineous purulent discharge from her right ear. After paraclinical and pathologic evaluation it was diagnosed as embryonic rhabdomyosarcoma. Conclusions. In all children with mastoiditis, especially in young children, rhabdomyosarcoma should be considered as a differential diagnosis.
    06/2012; 2012:871235. DOI:10.1155/2012/871235


Available from