IRSG Operations Office, Department of Pediatrics and Adolescent Medicine, Mayo 9-30E, Mayo Clinic, 200 First Street SW, Rochester, MN 55905-0001Search for more papers by this author

from the Intergroup Rhabdomyosarcoma Study Group (IRSG),

from the Intergroup Rhabdomyosarcoma Study Group (IRSG)

The Intergroup Rhabdomyosarcoma Study Group includes James R. Anderson, PhD, Richard J. Andrassy, MD, Carola A.S. Arndt, MD, K. Scott Baker, MD, Frederic G. Barr, MD, W. Archie Bleyer, MD, Philip Breitfeld, MD, John C. Breneman, MD, Julia Bridge, MD, Kenneth Brown, MD, William M. Crist, MD, Sarah S. Donaldson, MD, Holcombe E. Grier, MD, Douglas Hawkins, MD, Peter J. Houghton, PhD, Michael Link, MD, Thom L. Lobe, MD, Harold M. Maurer, MD, William H. Meyer, MD, Jeff Michalski, MD, Sharon Murphy, MD, Charles N. Paidas, MD, Alberto S. Pappo, MD, David M. Parham, MD, Stephen J. Qualman, MD, R. Beverly Raney, MD, Leslie Robison, PhD, Eric Sandler, MD, Lynn Smith, MD, Poul H.B. Sorensen, MD, PhD, Lisa Teot, MD, Timothy Triche, MD, PhD, Teresa J. Vietti, MD, David Walterhouse, MD, Moody Wharam, MD, Eugene S. Wiener, MD, Suzanne Wolden, MD, and Richard Womer, MD.

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R. Beverly Raney MD,

R. Beverly Raney MD

Children's Cancer Group, Arcadia, California

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

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James R. Anderson PhD,

James R. Anderson PhD

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

Intergroup Rhabdomyosarcoma Statistical Office, University of Nebraska, Omaha, Nebraska

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Jeffrey Kollath MS,

Jeffrey Kollath MS

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

Intergroup Rhabdomyosarcoma Statistical Office, University of Nebraska, Omaha, Nebraska

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Rena Vassilopoulou-Sellin MD,

Rena Vassilopoulou-Sellin MD

Section of Endocrinology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas

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Mary Jean Klein RN,

Mary Jean Klein RN

Section of Endocrinology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas

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Ruth Heyn MD,

Ruth Heyn MD

Children's Cancer Group, Arcadia, California

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

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Arvin S. Glicksman MD,

Arvin S. Glicksman MD

Quality Assurance Review Center, Dept. of Radiation Oncology, Roger Williams Cancer Center, Providence, Rhode Island

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Moody Wharam MD,

Moody Wharam MD

Pediatric Oncology Group, Chicago, Illinois

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William M. Crist MD,

William M. Crist MD

Children's Cancer Group, Arcadia, California

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

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Harold M. Maurer MD,

Corresponding Author

Harold M. Maurer MD

[email protected]

Children's Cancer Group, Arcadia, California

Intergroup Rhabdomyosarcoma Study Group, Mayo Clinic, Rochester, Minnesota

IRSG Operations Office, Department of Pediatrics and Adolescent Medicine, Mayo 9-30E, Mayo Clinic, 200 First Street SW, Rochester, MN 55905-0001Search for more papers by this author

from the Intergroup Rhabdomyosarcoma Study Group (IRSG),

from the Intergroup Rhabdomyosarcoma Study Group (IRSG)

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First published: 22 May 2000

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

Citations: 118

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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 reported 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. Med. Pediatr. Oncol. 34:413–420, 2000. © 2000 Wiley-Liss, Inc.

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Citing Literature

Volume34, Issue6

June 2000

Pages 413-420

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

Abstract

Background

Procedure

Results

Conclusions

REFERENCES

Citing Literature

References

Information

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

Abstract

Background

Procedure

Results

Conclusions

REFERENCES

Citing Literature

References

Related

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