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Photo Essay
May 2002

Primary Conjunctival Rhabdomyosarcoma

Arch Ophthalmol. 2002;120(5):668-669. doi:10.1001/archopht.120.5.668

A 4-YEAR-OLD girl was initially seen by us with a 2-month history of right upper eyelid ptosis and an enlarging superior forniceal conjunctival mass clinically consistent with a giant papilloma (Figure 1). Subtotal excision of the lesion was performed (Figure 2) and histopathological evaluation showed a botryoid type of embryonal rhabdomyosarcoma. A dense band of primitive, small round to spindle cells was present beneath the conjunctival epithelium consistent with a cambium layer, which is a criterion of the Intergroup Rhabdomyosarcoma Study for diagnosis of botryoid embryonal rhabdomyosarcoma (Figure 3).1 Scattered within the primitive cells were rhabdomyoblasts with eccentric nuclei and abundant eosinophilic cytoplasm (Figure 4). The immunohistochemical stainings for markers of myogenin (Figure 5) and desmin were strongly positive. The tumor was staged as clinical group II according to the criteria of the Intergroup Rhabdomyosarcoma Study.1 Postsurgical magnetic resonance imaging showed abnormal enhancement in the right palpebral fissure region (Figure 6). Treatment consisted of chemotherapy with vincristine sulfate and dactinomycin and fractionated radiotherapy to a total of 3600 rad (36 Gy). Six months after initiating treatment, the patient remains clinically tumor-free, with no ocular complications from radiation therapy.

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