Retinoblastoma is the most common intraocular tumor in
childhood. There is an increased risk of secondary malignancy
in these cases, especially in hereditary retinoblastoma. Here,
we report a case with bilateral retinoblastoma who was treated
with chemotherapy and radiotherapy, and developed soft tissue
sarcoma 14 years later within the radiation field. A 16-year-old
girl who had enucleation of the right eye and radiotherapy of
the left eye when she was 1 year old, presented with a mass
in the maxillary sinus. Biopsy was consistent with low-grade
non-rhabdomyosarcoma soft tissue sarcoma. The patient was
treated with surgery and chemotherapy, but had multiple recurrences.
She received various salvage chemotherapy regimens
and multiple surgical procedures, and is alive with disease.
There is an increased risk of secondary malignancy in
retinoblastoma, especially in the hereditary form. This risk is
increased in patients receiving radiotherapy. Tumors may appear
within the radiation field even years later.
Keywords: Secondary cancer; retinoblastoma; radiotherapy
