Clinical Experience of Unilateral Retinoblastoma

Unilateral retinoblastoma has revealed good prognosis after enucleation in long term follow-up period. But unilateral retinoblastoma with delayed diagnosis may result in poor prognosis due to invasion of the optic nerve or choroid or extraocular spread. Medical records of 26 patients(26 eyes) with unilateral retinoblastoma confirmed by biopsy result after enucleation, were reviewed at the Department of Ophthalmology, Yeungnam University College of Medicine from December, 1983 through February, 1998. Age of the patient, ocular findings, diagnostic methods, treatment modality and clinical results were analyzed. Age ranged from 1 to 60 months(average 26 months). Leukocoria was the most prominent ocular sign and retinal detachment or mass was observed by funduscopic examination in most cases. Enucleation was performed in all 26 cases. Chemotherapy and/or radiotherapy were carried out in 19 cases(73.1%). During the postoperative period of 7 months to 15 years(average 5.8 years), any of 26 eyes has not developed the second tumor, but distant metastases were observed in 4 eyes. The regions of distant metastasis were bone marrow, brain, combined bone marrow and brain, and abdomen. Chemotherapy and radiation therapy were performed in those 4 cases, yet 3 of them were died. Twenty-two patients who did not develop distant metastasis are still alive at the final follow-up.

Deletion of Rb1 gene in late osteosarcoma from survivor of unilateral retinoblastoma: a case report

It has been well known that the survivors of retinoblastoma are prone to have osteosarcoma. But the secondary tumor usually occurs in bilateral, hereditary type of retinoblastoma. We report one case of osteosarcoma in a survivor of unilateral, sporadic retinoblastoma. A fourteen year old male presented with a painfully swollen distal forearm of 2 month duration. He had enucleated his left eye 10 years ago due to retinoblastoma with no other adjuvant therapy. We managed him with our conventional protocol and identified deletion of Rb gene from his pathological specimen by using the PCR-RFLP method. This result is unusual for unilateral nonhereditable retinoblastoma and may suggest gene level change even in sporadic cases. And Rb gene study may be helpful for unilateral, sporadic retinoblastoma patient in detecting the possibility of late osteosarcoma.