title: Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation.
abstract: BACKGROUND
  There are no standardized diagnostic or treatment guidelines for patients with advanced unilateral retinoblastoma.
  
  
  METHODS
  Patients with advanced unilateral retinoblastoma were prospectively treated after enucleation using a risk-based protocol. Patients were assigned to low risk (LR), intermediate risk (IR), or high risk (HR) based on pathology. LR patients underwent observation. IR patients received 4 courses of chemotherapy with vincristine, doxorubicin, and cyclophosphamide (VDC). In the HR group, patients received 3 courses of VDC alternating with 3 courses of vincristine, carboplatin, and etoposide (VCE) and irradiation when indicated.
  
  
  RESULTS
  Fifty patients with advanced unilateral retinoblastoma were treated (LR, n=36; IR, n=7; HR, n=7). All eyes were Reese-Ellsworth group V. All bone scans (n=81), lumbar punctures (n=16), and bone marrow aspirates (n=16) were negative. Chemotherapy was well tolerated. Grades 3/4 hematologic toxicities were seen in all patients; grades 3/4 nonhematologic toxicities were seen in half the patients. Only one patient in the HR group received radiation therapy. All patients were alive at the time of analysis with no signs of disease recurrence. Median follow-up was 3.4 years (range, 0.8 to 6.4 y).
  
  
  CONCLUSIONS
  Patients with nonmetastatic unilateral retinoblastoma undergoing primary enucleation can be cured with a graduated intensity approach based on pathology.
authors.affiliation: *Departments of Oncology †Pathology ‡Surgery ∥Biostatistics ¶Radiological Sciences **Cancer Center Administration, St. Jude Children's Research Hospital §Departments of Ophthalmology #Pediatrics, University of Tennessee Health Sciences Center, Memphis, TN.
authors.forename: Erin M
authors.initials: EM
authors.lastname: Sullivan
authors.forename: Matthew W
authors.initials: MW
authors.lastname: Wilson
authors.forename: Catherine A
authors.initials: CA
authors.lastname: Billups
authors.forename: Jianrong
authors.initials: J
authors.lastname: Wu
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Merchant
authors.forename: Rachel C
authors.initials: RC
authors.lastname: Brennan
authors.forename: Barrett G
authors.initials: BG
authors.lastname: Haik
authors.forename: Barry
authors.initials: B
authors.lastname: Shulkin
authors.forename: Tammy M
authors.initials: TM
authors.lastname: Free
authors.forename: Vickie
authors.initials: V
authors.lastname: Given
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Qaddoumi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016190:Carboplatin / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003609:Dactinomycin / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D015353:Eye Enucleation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control; D011446:Prospective Studies; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000601:surgery*; D012175:Retinoblastoma / Q000188:drug therapy* / Q000601:surgery*; D018570:Risk Assessment / Q000379:methods; D013713:Teniposide / Q000627:therapeutic use; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide; D013713:Teniposide; D016190:Carboplatin