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B  CNS Extension
                                     1  Prechiasmatic lesion
                                     2  CNS mass
                                     3  Leptomeningeal disease


                       C)Treatment

                       A.   Intraocular  tumor,  International  Classification  Group  A  to  C,  Unilateral  or
                          Bilateral
                          Refer to retinoblastoma treatment center

                       B.   Intraocular tumor, International Classification Group D, Unilateral or Bilateral
                          Refer to retinoblastoma treatment center

                       C.   Intraocular tumor, International Classification Group E, Unilateral or Bilateral

                          1.  Primary enucleation with PMMA orbital implant
                              Special considerations for enucleation in retinoblastoma
                                Minimal manipulation
                                Avoid perforation of the eye
                                Harvest long (> 15 mm) optic nerve stump
                                Inspect  the  enucleated  eye  for  macroscopic  extraocular  extension  and
                                 optic nerve involvement

                                Harvest fresh tissue for genetic studies
                                Avoid biointegrated implant if postoperative radiotherapy is necessary

                          2.  Send  specimen  for  histopathology  and  high  risk  factors  predictive  of
                              metastasis
                                Anterior chamber seeding

                                Iris infiltration
                                Ciliary body infiltration
                                Massive choroidal infiltration
                                Invasion of the optic nerve lamina cribrosa
                                Retrolaminar optic nerve invasion
                                Invasion of optic nerve transection

                                Scleral infiltration
                                Extrascleral extension

                       D.   High risk factors on histopathology, International Staging, Stage II
                          Refer to retinoblastoma treatment center


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