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

Background. We reviewed the late complications of therapy in 94 patients wi th localized, primary rhabdomyosarcoma of the orbit treated on the Intergro up Rhabdomyosarcoma Study (IRS)-III protocol (1984-1991). Procedure. A ques tionnaire was sent to the institutions that had registered 106 patients wit h orbital RMS on the IRS-III protocol, seeking information about vision, pe riocular structures, and growth and development of the 102 survivors. Resul ts. Ninety-four questionnaires were returned. The median follow-up interval was 7.6 years. The affected eye was removed from 13 patients because of lo cal 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%) und erwent cataract surgery. Fifty five patients (70%) had decreased visual acu ity. Twenty-four patients had a dry eye, and 22 had chronic keratitis, conj unctivitis, or corneal changes. Strabismus, diplopia, retinopathy, and uvei tis were uncommon. The orbit was hypoplastic in 48 of 82 patients assessed (59%). Ptosis and enophthalmos were reported in 22 patients. Decreased stat ural growth was noted in 13 of the 53 irradiated patients aged 3-14 years a t diagnosis with sufficient data (24%). Conclusions. The overall survival r are was 96% (102/106). The eye was preserved in 86% of the patients, but vi sion was impaired in 70% of them. Other frequent complications were catarac t, orbital hypoplasia, keratoconjunctivitis, and ptosis/enophthalmos. The c urrent IRS-V study recommends decreasing the dose of irradiation and using conformal techniques in an attempt to minimize these complications. (C) 200 0 Wiley-Liss. Inc.