Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation

Erin M. Sullivan, Matthew W. Wilson, Catherine A. Billups, Jianrong Wu, Thomas E. Merchant, Rachel C. Brennan, Barrett G. Haik, Barry Shulkin, Tammy M. Free, Vickie Given, Carlos Rodriguez-Galindo, Ibrahim Qaddoumi

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

BACKGROUND:: There are no standardized diagnostic or treatment guidelines for patients with advanced unilateral retinoblastoma. MATERIALS AND 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.

Original languageEnglish
Pages (from-to)e335-e340
JournalJournal of Pediatric Hematology/Oncology
Volume36
Issue number6
DOIs
StatePublished - Aug 2014

Keywords

  • graduated
  • metastatic
  • retinoblastoma
  • therapy
  • unilateral

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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