Table 1
Clinical summary of the patients
| Patients (age) | Clinicoradiological features | Neoadjuvant treatment | Evaluation | Enucleation histology | Postoperative treatment | Post-treatment response |
|---|---|---|---|---|---|---|
| *Etoposide 100 mg/m2/day, D1–D5, carboplatin 160 mg/m2/day, D1–D5.2 | ||||||
| †CADO: cyclophosphamide 300 mg/m2/day, D1–D5, vincristine 1.5 mg/m2/day, D1, D5, adriamycin 30 mg/m2/day, D4, D5).9 | ||||||
| ‡Vincristine 1.5 mg/m2/day, D1, D5 cyclophosphamide 1 g/m2/day, D1–D3.9,10 | ||||||
| §Etoposide 150 mg/m2/day, D1–D3, cyclophosphamide 1 g/m2/day, D1–D3.8 | ||||||
| ¶CARBOPEC carboplatin 350 mg/m2/day, D1–D5, etoposide 350 mg/m2/day, D1–D5, cyclophosphamide 1.6 g/m2/day, D2–D5.11 | ||||||
| 1 (3.5 years) | Buphthalmia | 1 etoposide-carboplatin | Clinical: regression | Necrotic tumour Invasion: prelaminar, choroidal, ciliary bodies | 2 etoposide-carboplatin* 2 cado† | 21 months Cr |
| 2 (22 months) | Buphthalmia | 1 etoposide-carboplatin | Clinical: regression | Necrotic tumour Invasion: intralaminar | 1 etoposide-carboplatin 2 vincristine-endoxan‡ | 15 months Cr |
| 3 (22 months) | Orbital portion of optic nerve | 3 etoposide-carboplatin | CT/MRI: tumour reduction CSF (−) | Necrotic tumour No residual optic nerve invasion | 2 etoposide-cyclophosphamide§ CARBOPEC¶ | 40 months Cr |
| 4 (1 year) | Orbital portion of optic nerve | 3 etoposide-carboplatin 1 etoposide-cyclophosphamide | CT/MRI: tumour reduction CSF (−) | Necrotic tumour Invasion: prelaminar | 3 etoposide-carboplatin 1 etoposide-cyclophosphamide CARBOPEC | 36 months Cr |
| 5 (8 years) | Prechiasmatic portion of optic nerve | 2 etoposide-carboplatin Intrathecal thiotepa | MRI: tumour reduction CSF (−) | Macroscopic invasion of optic nerve resection margins Necrotic tumour | 3 etoposide-cyclophosphamide 1 etoposide-arboplatin CARBOPEC Radiotherapy | 12 months CR |
| 6 (18 months) | Buphthalmia Prechiasmatic portion of optic nerve | 2 etoposide-carboplatin | CT: tumour reduction CSF (+) | No enucleation Early meningeal progression | Died | |
