title: Chemoreduction for group E retinoblastoma: comparison of chemoreduction alone versus chemoreduction plus low-dose external radiotherapy in 76 eyes.
abstract: OBJECTIVE
  To evaluate chemoreduction (CRD) for group E retinoblastoma.
  
  
  METHODS
  Retrospective, comparative case series.
  
  
  METHODS
  Seventy-six eyes of 56 patients with group E retinoblastoma were treated with CRD alone or CRD plus low-dose prophylactic external beam radiotherapy (CRD+P-EBR). The CRD included vincristine, etoposide, and carboplatin (6 cycles). The P-EBR was given routinely 2 months after completion of CRD at a suggested dose of 2600 cGy. Therapeutic EBR (T-EBR) was only given at the time of extensive tumor recurrence at a suggested dose of 3800 cGy.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  Globe salvage.
  
  
  RESULTS
  Of the 76 eyes, 64 received CRD alone and 12 received CRD+P-EBR. At the 2-year follow-up, globe salvage was achieved in 29 (53%) of 55 eyes in the CRD group and in 10 (91%) of 11 eyes in the CRD+P-EBR group. At 5 years, globe salvage was achieved in 20 (48%) of 42 eyes in the CRD group and in 4 (80%) of 5 eyes in the CRD+P-EBR group (P=0.347). Of the 64 eyes in the CRD group, 16 (25%) were salvaged with CRD alone and 13 (20%) with CRD+T-EBR, whereas 22 (34%) were enucleated after CRD alone and 13 (20%) were enucleated after CRD+T-EBR. Of the 12 eyes in the CRD+P-EBR group, 10 (83%) were salvaged with CRD+P-EBR, whereas 2 (17%) were enucleated and none required T-EBR. The median dose for T-EBR was 3800 cGy, and that for P-EBR was 2600 cGy. Eyes treated with CRD+P-EBR showed significantly less recurrence, leading to less chance of enucleation or therapeutic radiotherapy than that for CRD alone (P<0.001). Visual acuity was 20/100 or better or fix and follow in 9 (32%) of 28 salvaged eyes in the CRD group and in 4 (40%) of 10 in the CRD+P-EBR group. At 5 years, there were no patients in either group with metastasis of pinealoblastoma or who had died. In one patient in the CRD group, a second cancer developed.
  
  
  CONCLUSIONS
  Group E retinoblastoma managed with CRD+P-EBR showed significantly less need for enucleation or therapeutic radiotherapy than eyes treated with CRD alone. These findings merit further study and consideration.
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Aparna
authors.initials: A
authors.lastname: Ramasubramanian
authors.forename: Archana
authors.initials: A
authors.lastname: Thangappan
authors.forename: Kimberly
authors.initials: K
authors.lastname: Hartzell
authors.forename: Ann
authors.initials: A
authors.lastname: Leahey
authors.forename: Anna T
authors.initials: AT
authors.lastname: Meadows
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005047:Etoposide / Q000627:therapeutic use; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local; D011882:Radiotherapy, High-Energy; D019572:Retinal Neoplasms / Q000145:classification / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D012175:Retinoblastoma / Q000145:classification / Q000188:drug therapy / Q000532:radiotherapy / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use; D014792:Visual Acuity
chemical_list: D014750:Vincristine; D005047:Etoposide; D016190:Carboplatin