Abstract
A 58-year-old man developed a left orbital mass 57 years after bilateral enucleation and external beam radiotherapy for bilateral sporadic retinoblastoma. Eighteen years earlier, the patient had a basal cell carcinoma successfully excised from the same area. Orbital computed tomography revealed an infiltrative soft tissue mass measuring 50 ± 40 ± 15 mm involving the left medial orbit, left ethmoid sinus and the cribriform plate. An incisional biopsy was performed, and the lesion proved to be a poorly differentiated rhabdomyosarcoma of alveolar cell type. Despite aggressive chemotherapy, intracranial extension progressed and the patient expired.Late second malignancy following irradiated and non-irradiated retinoblastoma has been documented to occur even up to 54 years after retinoblastoma diagnosis. The 57-year interval between retinoblastoma and second cancer highlights this phenomenon and emphasizes the need for long term medical care of these patients.
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