Background: Metastatic spread in retinoblastoma is a rare occurrence in developed countries but still associated with a poor prognosis.
Patients and methods: Medical records of all metastatic retinoblastoma diagnosed during a 20-year period were retrospectively reviewed.
Results: 6 patients out of 104 presented a metastatic disease with an overall occurrence of 5.8%. Three had a metastatic disease at diagnosis, one patient a trilateral retinoblastoma and 2 a metastatic spread after enucleation. All but one were sporadic retinoblastoma. Central Nervous System (CNS) involvement was reported in 5 patients while one patient had an intra-orbital lesion and bone and bone marrow spread. Different treatment strategies were administered based on local treatment plus chemotherapy and radiotherapy with or without high dose chemotherapy. Ifosfamide/carboplatin/etoposide (ICE) regimen was administered in 3 patients resulting in a partial response. Out of 6 patients, 4 died and 2 patients are alive at 60 and 63 months from diagnosis. Both children with a long follow-up were treated with high dose chemotherapy. All but one patients with CNS involvement died; the survivor was a patient with pineal involvement.
Conclusion: This retrospective review confirms a curable strategy based on local treatment and conventional plus high dose chemotherapy. Patients with CNS involvement remain incurable.