title: Periocular topotecan for intraocular retinoblastoma.
abstract: OBJECTIVE
  To review the effectiveness and toxicity of periocular topotecan hydrochloride in fibrin sealant (Tisseel) for the control of intraocular retinoblastoma.
  
  
  METHODS
  Retrospective medical record review of visually threatening or recurrent intraocular retinoblastoma treated with periocular topotecan.
  
  
  RESULTS
  Eight children (10 eyes) received 1 to 4 injections of periocular topotecan in fibrin sealant, without or with concomitant laser and/or single freeze-thaw prechemotherapy cryotherapy. Median dose was 0.18 mg/kg (3.72 mg/m(2)). The 6 children who responded to treatment had small discrete tumors (8 International Intraocular Retinoblastoma Classification group A or B eyes). Of these, prior primary treatment for 3 children (3 eyes) was laser; for 1 child (2 eyes), systemic chemotherapy with focal laser; and for 2 children (3 eyes), periocular topotecan. In 4 children (4 eyes), tumor regression was sufficient for effective focal therapy, but in 2 children (4 eyes), long-term control required systemic chemotherapy. The 2 children who did not respond each had an International Intraocular Retinoblastoma Classification group D eye treated primarily with systemic chemotherapy, focal laser, and cryotherapy and recurrent disease that was not controlled by periocular topotecan; both eyes were eventually enucleated. No ocular and minimal hematological toxic effects were observed. At 11 months' median follow-up after topotecan treatment (18 months since diagnosis), all 8 group A and B eyes were retained with ongoing focal therapy required in only 1 group B eye; the 2 group D eyes were enucleated.
  
  
  CONCLUSIONS
  Periocular topotecan in fibrin sealant can achieve volume reduction of small and recurrent retinoblastoma sufficient to allow successful focal therapy.
authors.affiliation: Departments of Ophthalmology and Visual Sciences, The Hospital for Sick Children, Ontario, Canada.
authors.forename: Ashwin C
authors.initials: AC
authors.lastname: Mallipatna
authors.forename: Helen
authors.initials: H
authors.lastname: Dimaras
authors.forename: Helen S L
authors.initials: HS
authors.lastname: Chan
authors.forename: Elise
authors.initials: E
authors.lastname: Héon
authors.forename: Brenda L
authors.initials: BL
authors.lastname: Gallie
mesh_terms: D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007267:Injections; D009915:Orbit; D012160:Retina / Q000187:drug effects / Q000473:pathology*; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000473:pathology; D012175:Retinoblastoma / Q000188:drug therapy* / Q000473:pathology; D012189:Retrospective Studies; D059004:Topoisomerase I Inhibitors / Q000008:administration & dosage*; D019772:Topotecan / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D059004:Topoisomerase I Inhibitors; D019772:Topotecan