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Ophthalmic involvement as a presenting feature of nonorbital childhood parameningeal embryonal rhabdomyosarcoma
P B Mullaney1, N U Nabi, P Thorner
1Divisions of Ophthalmology and Pathology, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
Insights
Ophthalmic signs like sixth nerve palsies in children may indicate advanced parameningeal rhabdomyosarcoma. Early detection is crucial, as these signs are often associated with a poorer prognosis and delayed treatment.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Neurology
Background:
- Parameningeal rhabdomyosarcoma is a rare but aggressive pediatric cancer.
- Ophthalmic manifestations can be the initial presenting signs in some cases.
- Timely diagnosis is critical for improving patient outcomes.
Purpose of the Study:
- To characterize the ophthalmic signs and symptoms observed at presentation in children diagnosed with parameningeal rhabdomyosarcoma.
- To correlate ophthalmic findings with tumor extent, treatment, and survival rates.
Main Methods:
- Retrospective case series of 23 children treated for parameningeal rhabdomyosarcoma.
- Analysis of presenting ophthalmic signs, tumor location via CT scans, pathology, and patient outcomes.
- Review of treatment protocols including surgery, chemotherapy, and radiotherapy.
Main Results:
- Eight of 23 patients presented with ophthalmic signs, primarily sixth nerve palsies.
- Additional cranial nerve palsies (third, fourth, fifth, seventh) and optic nerve involvement leading to vision loss were observed.
- Patients with ophthalmic signs had shorter survival times compared to those without.
Conclusions:
- Advanced parameningeal rhabdomyosarcoma invading the skull base can manifest with ophthalmic signs.
- Delayed presentation and diagnosis are common due to initial misattribution of symptoms.
- Ophthalmic involvement signifies a poorer prognosis in pediatric parameningeal rhabdomyosarcoma.
Objective:
To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma.
Design:
Retrospective, noncomparative case series.
Participants:
Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998.
Main Outcome Measures:
Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy.
Results:
Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months.
Conclusions:
Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.
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