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Diencephalic Tumor: a rare coincidence in retinoblastoma survivors?
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  • Maria Antonietta De Ioris,
  • Ida Russo,
  • Paola Valente,
  • Giovanna Stefania Colafati,
  • Iside Alessi,
  • Antonino Romanzo,
  • Luca Buzzonetti,
  • Angela Mastronuzzi,
  • Andrea Carai
Maria Antonietta De Ioris
Ospedale Pediatrico Bambino Gesù
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Ida Russo
Ospedale Pediatrico Bambino Gesu
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Paola Valente
Bambino Gesù Children's Hospital
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Giovanna Stefania Colafati
Ospedale Pediatrico Bambino Gesu
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Iside Alessi
Bambino Gesu Pediatric Hospital
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Antonino Romanzo
Bambino Gesù IRCCS Children's Hospital
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Luca Buzzonetti
Bambino Gesù IRCCS Children's Hospital
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Angela Mastronuzzi
Ospedale Pediatrico Bambino Gesù
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Andrea Carai
Bambino Gesù Children's Hospital
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Abstract

Retinoblastoma(RB) patients have a high risk to develop second malignant neoplasm (SMN). Diencephalic tumors(DT) are rare in paediatric age. A retrospective review was performed over 21 years period. Out of 169 RB patients, 3 presented a DT. Two patients presented a Rb1 germline mutation and none received radiotherapy. DT in previously treated RB patients seems a peculiar SMN. However, considering the site, the short time interval from RB and the absence of radiotherapy, an alternative pathogenic mechanism could be supposed. The same embryological origin of the retina and the diencephalon should be considered and biological studies are needed.