Delayed metastasis in patients with intraocular retinoblastoma: A review of three cases.

Abstract

To discuss the clinical presentation, management, and outcome of delayed metastasis in retinoblastoma (RB). Retrospective case series of three patients. Mean age at diagnosis of RB was 29 months (median, 28 months; range, 11-48 months). All were males with non-familial bilateral intraocular RB. Primary treatment for RB included intravenous chemotherapy in all three cases. Secondary treatment included transpupillary thermotherapy/cryotherapy (n = 6 eyes), periocular chemotherapy (n = 2 eyes), intravitreal chemotherapy (n = 1 eye), intra-arterial chemotherapy (n = 1 eye), external beam radiotherapy (EBRT; n = 2 eyes), and enucleation (n = 2 eyes). Primary tumor regression was achieved in all cases and remained status quo at the time of diagnosis of distant metastasis. Two patients developed bone metastasis (ulna; tibia) and one developed soft tissue metastasis (temporal fossa) over a mean follow-up period of 6 years (median, 7 years; range, 5-8 years) from diagnosis of RB. Mean age of detection of metastatic disease was 8 years (median, 8 years; range, 7-9 years). All the lesions were solitary and the diagnosis of metastatic retinoblastoma was confirmed by tissue biopsy. Metastatic disease was treated with surgical excision (n = 1), chemotherapy (n = 2), and EBRT (n = 2). All patients are alive, with two patients free of disease over a mean follow-up period of 23 months (median, 23 months; range, 12-33 months); and 1 in remission 7 months after completion of EBRT. Long-term follow-up of RB cases is mandatory. In spite of intraocular tumor regression, metastasis can still occur many years after treatment of RB.