Presentation, Prognostic Factors and Patterns of Failure in Adult Rhabdomyosarcoma

William Levin, MD
University of Pennsylvania Cancer Center
Last Modified: November 7, 2001

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Presenter: J.H. Simon
Presenter's Affiliation: University of Iowa, Iowa City
Type of Session: Scientific

Background
Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood, with about 400 new cases per year. Five year survival rates are around 70%. Unfortunately, adults with rhabdomyosarcoma have a much poorer prognosis. This retrospective study was undertaken to identify the factors responsible for poor outcome.

Materials and Methods

  • The cases of 39 patients were examined, 26 male and 13 female.
  • Median age was 45 years.
  • Multivariate analysis was performed using Cox regression models to determine prognostic factors for overall survival, progression free survival, and local control.

    Results

  • A bimodal age distribution was observed, with the first peak between the late teens-30 years and the second between 60-69 years.
  • 40% had extremity sarcomas.
  • 2/3 of tumors were larger than 5 cm.
  • Embryonal histology was found in 26 %.
  • Median survival was 2.25 years with an overall 5-year survival rate of 35 %.
  • Multivariate analysis identified non-embryonal histology, favorable site, female gender, early T stage, and absence of nodal metastasis as predictors of improved survival.
  • 5 year local control rate was 51 %.
  • Only early T-stage predicted for better local control.

    Author's Conclusions
    Overall prognosis of adult rhabdomyosarcoma in adults is worse than reported in children. Age criteria, within the adult population did not further classify outcome. Adult disease is more common in the extemities, while more common in the head and neck region in children.

    Clinical/Scientific Implications
    Generalizations from this study are not easily made, given that this is a small retrospective study from a single institution. Nevertheless, adults with rhabdomyosarcoma appear to fare less well than their pediatric counterparts

    Oncolink's ASTRO Coverage made possible by an unrestricted Educational Grant from Bristol-Myers Squibb Oncology and Pharmacia Oncology.


    News
    Survival Poor in Adults with Rhabdomyosarcoma

    Oct 23, 2014 - Adults with rhabdomyosarcoma, though rare, have significantly worse long-term survival than children with the disease, although many of the same factors predict survival in both cases, according to a study published online April 27 in the Journal of Clinical Oncology.



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