- Annexes
The international Retinoblastoma Staging System(1-3)(13)
Stage 0:
• Eye has not been enucleated and no dissemination of disease.
Stage I:
• Eye enucleated, completely resected histologically.
Stage II:
• Eye enucleated, microscopic residual tumor.
Stage III:
• Regional extension
III A: Overt orbital disease
III B: Preauricular or cervical lymph node extension
Stage IV:
• Metastatic disease.
IVa: Hematogenous metastasis (without CNS involvement)
IVa1: Single lesion
IVa2: Multiple lesions
IVb: CNS extension (with or without any other site of regional or metastatic disease)
IVb1: Prechiasmatic lesion
IVb2: CNS mass
IVb3: Leptomeningeal and CSF disease
High risk pathology features (1-3) (13)
• The main high-risk feature is extension of the tumour in the optic nerve to the margin of the surgical resection.
• Post laminar optic nerve invasion;
• Involvement of anterior segment;
• Massive invasion of choroid > 3mm;
• Invasion of sclera;
• Extra scleral tumour extension.
• Any degree of concomitant choroid and optic nerve involvement.