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Metastatic retinoblastoma: 20-year single institution experience
  1. Raffaele Cozza (cozza{at}opbg.net),
  2. Maria Antonietta De Ioris (deioris{at}opbg.net),
  3. Ilaria Ilari (ilari{at}opbg.net),
  4. Rita Devito (devito{at}opbg.net),
  5. Paola Fidani,
  6. Luigi De Sio (desio{at}opbg.net),
  7. Francesca Demelas (francesca.demelas{at}opbg.net),
  8. Antonino Romanzo (antoninoromanzo{at}hotmail.com),
  9. Alberto Donfrancesco (donfrancesco{at}opbg.net)
  1. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  2. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  3. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  4. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  5. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  6. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  7. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  8. Ospedale Pediatrico Bambino Gesù IRCCS, Italy
  9. Ospedale Pediatrico Bambino Gesù IRCCS, Italy

    Abstract

    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.

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