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Trans Am Ophthalmol Soc. 2001;99:133-42; discussion 142-3.

Primary ophthalmic rhabdomyosarcoma in 33 patients.

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Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.



To review the findings, management, and outcome in 33 cases of primary ophthalmic rhabdomyosarcoma.


The records of 33 consecutive patients from a single ocular oncology center were analyzed retrospectively for outcomes of final visual acuity, local recurrence, and distant metastasis.


Rhabdomyosarcoma was primarily located in the orbit m 25 cases (76%), conjunctiva in 4 (12%), eyelid in 1 (3%), and uveal tract in 3 (9%). Findings had been present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 (21%), and blepharoptosis in 6 (18%). The initial diagnoses before referral to us included primarily rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 (15%), cellulitis in 5 (15%), and pseudotumor in 4 (12%). Tumors were classified according to the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols as group I in 4 cases (12%), group II in 12 (36%), group III in 16 (48%), and group IV in 1 case (3%). Treatment included surgical debulking, chemotherapy, and radiotherapy. Local tumor recurrence was detected in 6 patients (18%), lymph node spread in 2 (6%), and distant metastasis in 2 (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 (18%), and 20/200 or worse in 12 (43%). Mean follow-up was 8.3 years; tumor-related death occurred in 1 patients (3%).


Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. Following treatment, local tumor recurrence occurs in 18% of cases, metastasis in 6%, and death in 3%.

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