Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984-1991

R. Beverly Raney, James R. Anderson, Jeffrey Kollath, Rena Vassilopoulou-Sellin, Mary Jean Klein, Ruth Heyn, Arvin S. Glicksman, Moody Wharam, William M. Crist, Harold M. Maurer

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Background. We reviewed the late complications of therapy in 94 patients with localized, primary rhabdomyosarcoma of the orbit treated on the Intergroup Rhabdomyosarcoma Study (IRS)-III protocol (1984-1991). Procedure. A questionnaire was sent to the institutions that had registered 106 patients with orbital RMS on the IRS-III protocol, seeking information about vision, periocular structures, and growth and development of the 102 survivors. Results. Ninety-four questionnaires were returned. The median follow-up interval was 7.6 years. The affected eye was removed from 13 patients because of local recurrence (N = 10) or other causes (N = 3). Seventy-nine of the eighty-one remaining patients had received radiation therapy. Sixty-five of these seventy-nine patients (82%) developed a cataract, and 43 of them (66%) underwent cataract surgery. Fifty-five patients (70%) had decreased visual acuity. Twenty-four patients had a dry eye, and 22 had chronic keratitis, conjunctivitis, or corneal changes. Strabismus, diplopia, retinopathy, and uveitis were uncommon. The orbit was hypoplastic in 48 of 82 patients assessed (59%). Ptosis and enophthalmos were re ported in 22 patients. Decreased statural growth was noted in 13 of the 53 irradiated patients aged 3-14 years at diagnosis with sufficient data (24%). Conclusions. The overall survival rate was 96% (102/106). The eye was preserved in 86% of the patients, but vision was impaired in 70% of them. Other frequent complications were cataract, orbital hypoplasia, keratoconjunctivitis, and ptosis/enophthalmos. The current IRS-V study recommends decreasing the dose of irradiation and using conformal techniques in an attempt to minimize these complications. (C) 2000 Wiley-Liss, Inc.

Original languageEnglish (US)
Pages (from-to)413-420
Number of pages8
JournalMedical and Pediatric Oncology
Issue number6
StatePublished - Jun 1 2000



  • Childhood cancer
  • Late complications
  • Orbit
  • Rhabdomyosarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

Cite this

Raney, R. B., Anderson, J. R., Kollath, J., Vassilopoulou-Sellin, R., Klein, M. J., Heyn, R., Glicksman, A. S., Wharam, M., Crist, W. M., & Maurer, H. M. (2000). Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984-1991. Medical and Pediatric Oncology, 34(6), 413-420.<413::AID-MPO6>3.0.CO;2-4