Second Malignancies After Long-Term Follow-Up in Retinoblastoma Treated With Chemoreduction

Abstract

Purpose: : To evaluate the long-term occurrence of second non-ocular malignancies (SM) in retinoblastoma (RB) patients treated with chemoreduction (vincristine, etoposide, carboplatin for 6 cycles)

Methods: : Retrospective chart review of 272 retinoblastoma patients

Results: : At a mean follow-up of 10 years, SM occurred in 6 (2.2%) of all 272 patients and 6 (3.3%) of 181 germline mutation patients. The mean age at the diagnosis of SM was 113 months. All 6 patients displayed bilateral sporadic retinoblasytoma and confirmed RB1 germline mutation. Four patients had additional ocular external beam radiotherapy (4367 cGy) and the SM occurred in the radiation field in 3 cases and in the head and neck area in all 4 cases. The SM included osteosarcoma (femur (n=1), orbit bone (n=2)), leiomyosarcoma (urinary bladder (n=1)), acute myeloid leukemia (AML (n=1) and one patient with both conjunctival melanoma and later rhabdomyosarcoma (temporal fossa). The mean interval between chemoreduction initiation and SM was 98 months and external ocular radiotherapy to SM was 93 months. One patient died following orbital osteosarcoma with brain invasion.

Conclusions: : At minimum 10 year follow-up of retinoblastoma patients treated with chemoreduction, SM occurred in 2.2% of patients, only those with germline mutation and generally those who received additional radiotherapy.

Keywords: retina • tumors • retinoblastoma