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 with localized, primary rhabdomyosarcoma of the orbit treated on the Intergroup Rhabdomyosarcoma Study (IRS)-III protocol (1984-1991).


METHODS
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.
Raney
R B
RB
Children's Cancer Group, Arcadia, California, USA.
Anderson
J R
JR
Kollath
J
J
Vassilopoulou-Sellin
R
R
Klein
M J
MJ
Heyn
R
R
Glicksman
A S
AS
Wharam
M
M
Crist
W M
WM
Maurer
H M
HM
D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001827:Body Height / Q000528:radiation effects; D002386:Cataract / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005128:Eye Diseases / Q000209:etiology*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D009915:Orbit / Q000528:radiation effects; D009918:Orbital Neoplasms / Q000188:drug therapy / Q000532:radiotherapy*; D011832:Radiation Injuries / Q000209:etiology*; D011878:Radiotherapy / Q000009:adverse effects; D011879:Radiotherapy Dosage; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy*; D011795:Surveys and Questionnaires; D016019:Survival Analysis
D000970:Antineoplastic Agents