Table 2.
Staging, treatment, and causes of death for 24 Retinoblastoma patients who died in our series.
| Characteristics (total 24 patients) | Number (%) | |
|---|---|---|
| Stage of the worst eye (IIRC)a | A,B,C | 1 (4%) |
| D | 6 (25%) | |
| E | 15 (63%) | |
| Extraocular | 2 (8%) | |
| Stage of the worst eye (TNM) | T1 | 0 (0%) |
| T2 | 7 (29%) | |
| T3 | 15 (63%) | |
| T4 | 2 (8%) | |
| Treatment modalities | Primary enucleation | 13 (59%) |
| Primary enucleation (refused)c | 4 (17%) | |
| Secondary enucleation | 5 (21%) | |
| Neoadjuvent systemic chemotherapy | 14 (60%) | |
| EBRTe | 8 (33%) | |
| High-risk features (for 18 enucleated eyes) | No | 4 (17%) |
| Optic nerve (post laminar) | 6 (25%) | |
| Choroid (massive) | 7 (29%) | |
| Anterior chamber/ ciliary body | 2 (8%) | |
| Cause of death | Metastasisb | 21 (88%) |
| Second malignancyd | 3 (12%) | |
| Site of metastasis (21 patients) | CNS | 11 (52%) |
| Bone marrow | 7 (33%) | |
| Multi organ metastasis | 3 (14%) | |
| Median time between diagnosis and death | 34 months | |
| Median time between metastasis and death | 6 months | |
a
IIRC, International Intraocular Retinoblastoma Classification.
b
One had metastatic pineoblastoma.
c
The primary treatment plan for these 4 patients was enucleation but parents refused decision and came back later on with extraocular disease.
d
Three patients had second malignancy (1 osteosarcoma in the thigh, 1 osteosarcoma in the orbit, and 1 liposarcoma in the back).
e
EBRT, External beam radiation therapy.