Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation

Erin M. Sullivan, Matthew 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 journalArticle

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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 (US)
JournalJournal of pediatric hematology/oncology
Volume36
Issue number6
DOIs
StatePublished - Jan 1 2014

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Retinoblastoma
Adjuvant Chemotherapy
Vincristine
Doxorubicin
Cyclophosphamide
Pathology
Drug Therapy
Spinal Puncture
Carboplatin
Etoposide
Radiotherapy
Bone Marrow
Observation
Guidelines

All Science Journal Classification (ASJC) codes

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

Cite this

Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation. / Sullivan, Erin M.; Wilson, Matthew; Billups, Catherine A.; Wu, Jianrong; Merchant, Thomas E.; Brennan, Rachel C.; Haik, Barrett G.; Shulkin, Barry; Free, Tammy M.; Given, Vickie; Rodriguez-Galindo, Carlos; Qaddoumi, Ibrahim.

In: Journal of pediatric hematology/oncology, Vol. 36, No. 6, 01.01.2014.

Research output: Contribution to journalArticle

Sullivan, EM, Wilson, M, Billups, CA, Wu, J, Merchant, TE, Brennan, RC, Haik, BG, Shulkin, B, Free, TM, Given, V, Rodriguez-Galindo, C & Qaddoumi, I 2014, 'Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation', Journal of pediatric hematology/oncology, vol. 36, no. 6. https://doi.org/10.1097/MPH.0000000000000141
Sullivan, Erin M. ; Wilson, Matthew ; Billups, Catherine A. ; Wu, Jianrong ; Merchant, Thomas E. ; Brennan, Rachel C. ; Haik, Barrett G. ; Shulkin, Barry ; Free, Tammy M. ; Given, Vickie ; Rodriguez-Galindo, Carlos ; Qaddoumi, Ibrahim. / Pathologic risk-based adjuvant chemotherapy for unilateral retinoblastoma following enucleation. In: Journal of pediatric hematology/oncology. 2014 ; Vol. 36, No. 6.
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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.",
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AU - Brennan, Rachel C.

AU - Haik, Barrett G.

AU - Shulkin, Barry

AU - Free, Tammy M.

AU - Given, Vickie

AU - Rodriguez-Galindo, Carlos

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N2 - 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.

AB - 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.

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