The aim of this study was to review the clinical presentation, management, local and systemic outcomes of 14 orbital rhabdomyosarcoma (OR) cases with the focus on the role of surgery as treatment. The mean age at presentation was eight years (three months – 12.8 years) with the primary location in the orbit. Proptosis was the most common clinical sign. Mean duration of symptoms was five weeks. Histopathological subtype was embryonal. Orbital CT scan in 11 cases showed extraconal homogenous mass that was noncalcified and with generalised contrast enhancement. Two patients had bone erosion and intracranial invasion. Treatment included surgical debulking, chemotherapy and radiotherapy. Orbital exenteration was required in four cases. Outcomes for 10 cases included: best corrected visual acuity of 20/20 to 20/40 in 60%, two patients with no light perception, seven with cataract, six with ocular surface lesions, two with orbital hypoplasis, four with ptosis, eight with ocular motility disorders, five with local tumour recurrence and two with distant metastasis (intracranial). Mean follow-up was 15.2 years with one tumour-related death. Characteristics were similar to the reported literature. The authors advocate initial extensive surgery with preservation of vital structures where possible.
Orbital rhabdomyosarcoma treatment characteristics
Reviewed by Fiona Rowe
Clinical management of orbital rhabdomyosarcoma in a referral center in Spain.
CONTRIBUTOR
Fiona Rowe (Prof)
Institute of Population Health, University of Liverpool, UK.
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