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 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.

Perioperative Intensity-Modulated Brachytherapy for Refractory Orbital Rhabdomyosarcomas in Children

“…Despite all advances, there is, however, still a significant overall number of therapy-related side effects impairing the quality of life in this young patient group [6,19,22,[36][37][38][39]49]. IMRT appears to offer a dosimetric advantage compared to conformal techniques [32].…”

“…The latter one may reflect increasing risk of eye dryness associated with doses of > 40 Gy [4]. The case of secondary leukemia corresponded to incidental findings of secondary neoplasm in other series of multimodally treated children with RMS and it was considered to be most probably chemotherapy-related in concordance with the literature [8,10,22,36,39].…”

Soft-Tissue sarcoma in childhood and adolescence

“…In another study, Raney et al [12] described late effects of therapy in 94 patients treated for localized RMS of the orbit on IRS-III from 1984 to 1991. The patients were assessed by local institutional physicians, and results were collected by questionnaire.…”

“…Overall, 59% of the patients had orbital hypoplasia, 32% had keratoconjunctivitis or corneal changes, 30% had dry eye, 28% had enophthalmos, and 24% had decreased growth. Compared with the IRS-I series of 50 patients with orbital RMS reported by Heyn et al [13], the number of patients with orbital tumors who developed growth failure was decreased, and the number undergoing cataract surgery was increased [12].…”

Management of sarcomas of the head and neck

“…Late complications in 213 patients from IRS II and III included facial asymmetry in almost one third, failure to maintain initial height velocity in one half, poor dentition in one fourth, and impaired vision, hearing, and learning (one sixth each) [36•]. Specifically, in those receiving treatment for RMS of the orbit-in almost all cases radiotherapy-80% developed a cataract, 70% had decreased visual acuity, and 59% had a hypoplastic orbit [37].…”