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Ocular manifestations and long-term complications of rhabdomyosarcoma in children
Sybille Graef1, Dan DeAngelis1, Abha A Gupta2
1Department of Ophthalmology and Vision Sciences, University of Toronto and Hospital for Sick Children, Toronto, ON, Canada.
Insights
Half of children with rhabdomyosarcoma present with ocular manifestations, often involving orbital structures. Cranial nerve involvement significantly worsens survival prognosis in pediatric rhabdomyosarcoma patients.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Head and Neck Surgery
Background:
- Rhabdomyosarcoma is a common pediatric malignancy.
- Ocular manifestations can be significant in head and neck rhabdomyosarcoma.
- Understanding these manifestations is crucial for diagnosis and management.
Purpose of the Study:
- To describe the ocular manifestations of rhabdomyosarcoma in a large pediatric cohort.
- To determine the incidence of ocular findings at presentation.
- To evaluate the prognostic role of ocular findings and long-term complications.
Main Methods:
- Retrospective observational cohort study.
- Analysis of medical records for pediatric patients with head and neck rhabdomyosarcoma (1997-2021).
- Assessment of ocular findings at presentation and long-term complications.
Main Results:
- 77 children with head and neck rhabdomyosarcoma were analyzed.
- 38 patients (49.4%) had ocular manifestations at presentation.
- Common findings included proptosis, motility restriction, strabismus, and ptosis. Long-term complications involved bony hypoplasia and keratopathy.
- Cranial nerve palsies were associated with significantly poorer survival (42% vs. 100% for primary orbital RMS).
Conclusions:
- Ocular manifestations are frequent in pediatric rhabdomyosarcoma.
- Orbital involvement and cranial nerve palsies impact prognosis.
- Early identification of ocular signs is vital for managing head and neck rhabdomyosarcoma.
Background/Objectives:
The purpose of the study was to describe the ocular manifestations of rhabdomyosarcoma in a large cohort of children.
Subject/Methods:
This was a retrospective observational cohort study. The medical records of all pediatric patients with head and neck rhabdomyosarcoma diagnosed between 1997 and 2021 at a tertiary-care pediatric hospital were analyzed. The main outcome measures were the incidence and prognostic role of ocular findings at presentation and long-term ocular complications.
Results:
There were 77 children with head and neck rhabdomyosarcoma in the study cohort with 38 patients showing ocular manifestations at presentation. Median age at diagnosis was 6.0 years, the median follow-up was 5.7 years and 54.5% were male. At last follow-up, 70.1% had no evidence of progression, 26.0% were deceased, and 2.6% were on palliative treatment. Orbital signs were common (44.2%). The most common ocular findings were proptosis (18.2%), restriction of extraocular motility (28.6%), strabismus/diplopia (22.1%) and ptosis (16.9%). The most common long-term complications were bony hypoplasia/facial asymmetry (40.3%) and keratopathy/dry eye (31.2%). Poor visual acuity (≤20/200) was noted in 13 (16.9%) patients with 5 (6.5%) patients requiring an exenteration. Survival was 100% in primary orbital RMS (p = 0.02), whereas any or a combination of cranial nerve palsies carried a poor prognosis (42% survival, p = 0.008).
Conclusions:
In our cohort, half of children with rhabdomyosarcoma had ocular manifestations at presentation with about one-third showing orbital tumor involvement. Cranial nerve involvement carried a significantly worse prognosis for survival.
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