Background: Outcome of patients with retinoblastoma is strongly related to the extent of the disease at diagnosis. Thus, prognosis of extra-ocular retinoblastoma is poor and metastases to central nervous system are usually fatal.
Case report: An 8-year-old child presented with a late recurrence of bilateral retinoblastoma. Initial treatment had associated enuclation of the left eye and radiation therapy on the right. At time of tumour recurrence, clinical examination showed cervical lymph nodes. There was a large frontal metastasis on the CT scan and bone marrow examination showed a minimal involvement by malignant cells. The patient achieved complete remission with high dose etoposide and carboplatin; he subsequently underwent massive chemotherapy followed by bone marrow rescue, and received additional radiation therapy on the frontal and cervical areas. He is well, five years after relapse.
Conclusions: This case highlights efficacy of chemotherapy in advanced retinoblastomas. Intensive chemotherapy is a promising and potentially curative approach in the management of advanced or metastatic retinoblastomas.