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

Research output: Contribution to journalArticle

99 Scopus citations

Abstract

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
Volume34
Issue number6
DOIs
StatePublished - Jun 1 2000
Externally publishedYes

Keywords

  • Childhood cancer
  • Late complications
  • Orbit
  • Rhabdomyosarcoma

ASJC Scopus subject areas

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

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    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. https://doi.org/10.1002/(SICI)1096-911X(200006)34:6<413::AID-MPO6>3.0.CO;2-4