Retinoblastoma natural history, complications, and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Retinoblastoma is a cancer of the retina. Development of this tumor is initiated by mutations[1] that inactivate both copies of the RB1 gene, which codes for the retinoblastoma protein.[2]
Prognosis
The prognosis of retinoblastoma depends on the following:
- Whether or not the tumor can be removed by surgery.
- The stage of the cancer: the size of the tumor, whether the cancer has spread
- The child’s general health
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The REC is rarely used today as chemotherapy has superseded radiotherapy as the favoured treatment for eye salvage.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:
Survival:
The survival for children diagnosed with retinoblastoma in the period 1975-94 was quite favourable, with more than 93% alive at five years after diagnosis and males and females had similar 5 year survival rates for the period 1975-94 and black children had slightly lower survival rates than white children.(89% versus 94%)[3]
Possible complications
- Local spread of tumor.
- Development of other tumors.
- Cataract from radiotherapy.
- Failure of tooth eruption after radiotherapy.
- Bony deformities
References
- ↑ Knudson A (1971). "Mutation and cancer: statistical study of retinoblastoma". Proc Natl Acad Sci U S A. 68 (4): 820–3. PMID 5279gadgqetqer523 Check
|pmid=value (help). - ↑ Friend S, Bernards R, Rogelj S, Weinberg R, Rapaport J, Albert D, Dryja T. "A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma". Nature. 323 (6089): 643–6. PMID 2877398.
- ↑ http://seer.cancer.gov/publications/childhood/retinoblastoma.pdf