title: [Retinoblastoma regression patterns and results following chemo reduction and adjuvant therapy].
abstract: OBJECTIVE
  To evaluate retinoblastoma regression patterns following chemo reduction and adjuvant therapy.
  
  
  METHODS
  Retrospective case series. 122 tumors of 47 eyes of 37 patients following chemo reduction and adjuvant therapy between January 2005 and June 2009 in the Eye & ENT hospital of Fudan University. Twenty-seven patients are male, and 10 patients are female. The average age was 22 months. The combined therapy included chemo reduction using vincristine, etoposide, and carboplatin (VEC) combined with local cryotherapy and/or transpupillary thermotherapy (TTT). The average follow-up duration was 32 months ranging from 12 to 60 months. Regression patterns included type 0 (no remnant), type 1 (calcified remnant), type 2 (noncalcified remnant), type 3 (partially calcified remnant), and type 4 (flat scar). Wilcoxon rank sum test was used to test the difference of tumor number between the patients with family history and those without family history. Chi-square test was used to test the difference between the tumor thickness, tumor location and regression patterns. Multivariate logistic regression analysis was used to test the correlation between the regression patterns and age, sex, tumor thickness, tumor location and family history. Statistical significance was assigned at P < 0.05.
  
  
  RESULTS
  Forty-seven eyes according to the International Intraocular Retinoblastoma Classification, 20 eyes (42.6%) were group A, 13 eyes (27.6%) group B, 6 eyes (12.8%) group C, 8 eyes (17.0%) group D. Of 122 tumors, the average number of tumors per eye was 2.6. Retinoblastoma regressions were type 0 (n = 3), type 1 (n = 15), type 2 (n = 8), type 3 (n = 25), and type 4 (n = 71). Tumor thickness and tumor location were related to regression patterns. Tumors with an initial thickness of 2 mm or less regressed most often to type 4, and those thicker than 8 mm regressed to type 1 or type 3. Tumors with greater distance from the foveola regressed most often to type 4. The factors predictive of regression pattern type 1 included tumor thickness larger than 8 mm (Z = 3.02, P = 0.003). The factors predictive of regression pattern type 3 included older age, tumor thickness larger than 8 mm and location not in the equator to ora serrata region (Z = 3.98, 2.23, 3.60; P = 0.000, 0.025, 0.000). The factors predictive of regression pattern type 4 included familial hereditary pattern, tumor thickness smaller than 2 mm and location in the equator to ora serrata region. (Z = 4.37, 3.42, 2.42; P = 0.000, 0.000, 0.021). 12 tumors recurred, 9 tumors were type 3 and 3 tumors were type 4. 8 eyes developed 15 new tumors. 5 patients developed new tumors were all younger patients and had familial hereditary history. The average period of recurrence of main tumors and development of new tumors was six months after the end of chemo reduction.
  
  
  CONCLUSIONS
  Following chemo reduction, type 3 and type 4 regression patterns were most common. Smaller tumors were usually seen in type 4, and bigger tumors were usually seen in type 1 or type 3. Tumor recurrence was usually found following regression pattern type 3 or type 4. Younger patients and patients with familial hereditary history trend to develop new tumors. Patients accept chemo reduction and adjuvant therapy need close follow-up.
authors.affiliation: Department of Ophthalmology, Eye & ENT Hospital of Fudan University, Shanghai, China.
authors.forename: Kang
authors.initials: K
authors.lastname: Xue
authors.forename: Jiang
authors.initials: J
authors.lastname: Qian
authors.forename: Han
authors.initials: H
authors.lastname: Yue
authors.forename: Yi-fei
authors.initials: YF
authors.lastname: Yuan
authors.forename: Rui
authors.initials: R
authors.lastname: Zhang
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols; D002648:Child; D002675:Child, Preschool; D017679:Cryotherapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019572:Retinal Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D012175:Retinoblastoma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome