Nan Li

and 8 more

Purpose: To investigate the clinical characteristics, treatment, and outcomes of metastatic and recurrent retinoblastoma in patients with stable primary tumors. Methods: Clinical data were reviewed for nine patients (two boys and seven girls; median age, 23 months) with stable primary tumors who exhibited metastatic and recurrent retinoblastoma. All patients were treated in the Pediatrics Department of our hospital in the past 5 years. The clinical characteristics, treatment methods, and patient prognoses were recorded. Results: Of the nine patients, three had binocular disease and six had monocular disease (n=12 eyes in this study). The median interval between the end of initial treatment and onset of recurrence was 12 months (range, 3–96 months). Of the nine patients, primary tumors were proved to be stable, seven had bone metastasis, five had lymph node metastasis, two had parotid gland involvement, and three had bone marrow infiltration. At the end of the study period, one patient was lost to follow-up, one patient died, seven patients were alive and three of them were in complete remission and survived disease-free until the end of follow-up. In December 2020, the median overall survival after onset was 32.0 months (range, 18–155 months); the median overall survival after recurrence was 18.0 months (range, 5–34 months). Conclusions: Recurrent and metastatic retinoblastomas are uncommon and easily misdiagnosed at an early stage in patients with stable primary tumors. These cancers can be effectively treated using systemic intravenous chemotherapy combined with surgery, radiotherapy, and hematopoietic stem cell transplantation.