METASTATIC RETINOBLASTOMA

Br J Ophthalmol. 2009 Jun 24. [Epub ahead of print]

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Metastatic retinoblastoma: 20-year single institution experience.

Cozza R, De Ioris MA, Ilari I, Devito R, Fidani P, De Sio L, Demelas F, Romanzo A, Donfrancesco A.

Italy.

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.