Retinoblastoma staging
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Risk calculators and risk factors for Retinoblastoma staging |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Staging
Reese-Ellsworth classification(REC) for Retinoblastoma:
The Reese-Ellsworh classification was developed in the 1960s by Dr.Algernon Reese and Dr.Robert Ellsworth, two prominent New York doctors specialising in retinoblastoma. The system was designed to predict outcome from treatment with External beam radiotherapy(EBRT), used internationally as the primary eye salvage treatment until intoduction of chemotherapy in the 1980s.
The REC is rarely used today as chemotherapy has superseded radiotherapy as the favoured treatment for eye salvage.
Group 1: Very favourable for maintenance of sight
A: Solitary tumor, smaller than 4 disc diameters(DD) at or behind the equator
B: Multiple tumors, none larger than 4 DD, all at or behind the equator
Group 2: Favourable for maintenance of sight
A: Solitary tumor, 4 to 10 DD at or behind the equator
B: Multiple tumors, 4 to 10 DD behind the equator
Group 3: possible for maintenance of sight
A: Any lesion anterior to the equator
B: Solitary tumor, larger than 10 DD behind the equator
Group 4: unfavourable for maintenance of sight
A: Multiple tumors, some larger than 10 DD
B: Any lesion extending anteriorly to the ora serrata
Group 5: very unfavourable for maintenance of sight
A: Massive tumors involving more than one half of the retina
B: Vitreous seeding
Essen classification:
International retinoblastoma classification:
| Stage 0: Patients treated conservatively (subject to presurgical ophthalmologic classifications) |
| Stage I: Eye enucleated, completely resected histologically |
| Stage II: Eye enucleated, microscopic residual tumor |
| Stage III: Regional extensiona |
| a) Overt orbital disease |
| b) Preauricular or cervical lymph node extension |
| Stage IV: Metastatic disease |
| a) Hematogenous metastasis: |
| 1. single lesion |
| 2. multiple lesions |
| b) CNS extension: |
| 1. Prechiasmatic lesion |
| 2. CNS mass |
| 3. Leptomeningeal disease |
The International Intraocular Retinoblastoma Classification(IIRC)or The ABC classification:
| Group A: small tumors away from foveola and disc |
| • Tumors <3 mm in greatest dimension confined to the retina and |
| • Located at least 3 mm from the foveola and 1.5 mm from the optic disc |
| Group B: all remaining tumors confined to the retina |
| • All other tumors confined to the retina and not in group A |
| • Subretinal fluid (without subretinal seeding) < 3 mm from the base of the tumor |
| Group C: local subretinal fluid or vitreous seeding |
| • Subretinal fluid alone >3 mm and < 6 mm from the tumor |
| • Vitreous or subretinal seeding < 3 mm from the tumor |
| Group D: diffuse subretinal fluid or seeding |
| • Subretinal fluid > 6 mm from the tumor |
| • Vitreous or subretinal seeding > 3 mm from the tumor |
| Group E: presence of any one or more of these poor prognosis features |
| • More than 2/3 of the globe filled with tumor |
| • Tumor in the anterior segment or anterior to the vitreous |
| • Tumor in or on the ciliary body |
| • Iris neovascularisation |
| • Neovascular glaucoma |
| • Opaque media from hemorrhage |
| • Tumor necrosis with aseptic orbital celullitis |
| • Phthisis bulbi |