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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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

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Rhabdomyosarcoma
Enophthalmos
Therapeutics
Cataract
Rhabdomyosarcoma of the orbit
Keratoconjunctivitis
Conjunctivitis
Diplopia
Keratitis
Strabismus
Uveitis
Orbit
Growth and Development
Visual Acuity
Survivors
Radiotherapy
Survival Rate
Recurrence

Keywords

  • Childhood cancer
  • Late complications
  • Orbit
  • Rhabdomyosarcoma

ASJC Scopus subject areas

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

Cite this

Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit : Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984-1991. / Raney, R. Beverly; Anderson, James R.; Kollath, Jeffrey; Vassilopoulou-Sellin, Rena; Klein, Mary Jean; Heyn, Ruth; Glicksman, Arvin S.; Wharam, Moody; Crist, William M.; Maurer, Harold M.

In: Medical and Pediatric Oncology, Vol. 34, No. 6, 01.06.2000, p. 413-420.

Research output: Contribution to journalArticle

Raney, R. Beverly ; Anderson, James R. ; Kollath, Jeffrey ; Vassilopoulou-Sellin, Rena ; Klein, Mary Jean ; Heyn, Ruth ; Glicksman, Arvin S. ; Wharam, Moody ; Crist, William M. ; Maurer, Harold M. / Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit : Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984-1991. In: Medical and Pediatric Oncology. 2000 ; Vol. 34, No. 6. pp. 413-420.
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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.",
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AU - Kollath, Jeffrey

AU - Vassilopoulou-Sellin, Rena

AU - Klein, Mary Jean

AU - Heyn, Ruth

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N2 - 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.

AB - 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.

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