Retinoblastoma natural history, complications, and prognosis: Difference between revisions
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In diffuse growth, there is relatively flat infiltration of the retina instead of a discrete mass. Diagnosis is very difficult and delayed. They produce a picture that may be mistaken for [[Vitritis]], [[Retinitis]] or [[Endophthalmitis]] | In diffuse growth, there is relatively flat infiltration of the retina instead of a discrete mass. Diagnosis is very difficult and delayed. They produce a picture that may be mistaken for [[Vitritis]], [[Retinitis]] or [[Endophthalmitis]] | ||
Spontaneous regression of the tumor is very rare and may occur due to unknown mechanism.(occlusion of the central retinal artery, severe inflammatory reaction and massive necrosis leading to [[pthisis bulbi]]) | Spontaneous regression of the tumor is very rare and may occur due to unknown mechanism.(occlusion of the central retinal artery, severe inflammatory reaction and massive necrosis leading to [[pthisis bulbi]]) | ||
Retinoblastoma is a rapidly growing tumor. If left untreated, the tumor fills the eye and completely destroys the globe in six months. Metastatic spread begins after six months and metastasized tumor is very rare at presentation. The tumor may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system or hematogenously to the lung, bone, or brain or by lymphatics if the tumor spreads anteriorly into the conjunctivae and eyelids, or extends into extraocular tissue. The most common routes of metastatic spread are direct infiltration via the optic nerve to the central nervous system, or spread via the choroid to the orbit | |||
Retinoblastoma is a rapidly growing tumor. If left untreated, the tumor fills the eye and completely destroys the globe in six months. Metastatic spread begins after six months and metastasized tumor is very rare at presentation. | |||
==Prognosis== | ==Prognosis== | ||
Revision as of 13:30, 29 May 2012
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]
Natural History
The growth form of the cancer can be exophytic, endophytic, mixed or diffuse.[3]
In the exophytic growth which occurs in the subretinal space is often associated with subretinal fluid accumulation and retinal detachment. The growth may infiltrate through the Bruch membrane(the innermost layer of the choroid) in to the choroid and then invade the ciliary nerves and blood vessels. The increase in caliber and tortuosity of retinal vessels which are noted in exophytic growth is because they lie over the mass. It usually results in proptosis and retinal detachment.
In the endophytic growth, the tumor breaks through the internal limiting membrane of retina and it is typically associated with vitreous seeding. There are no surface vessels in endophytic growth. It usually causes visual disturbance and white eye reflex.
The mixed growth pattern is the most common growth pattern and cobine the clinical features of both exophytic and endophytic growth patterns.
In diffuse growth, there is relatively flat infiltration of the retina instead of a discrete mass. Diagnosis is very difficult and delayed. They produce a picture that may be mistaken for Vitritis, Retinitis or Endophthalmitis
Spontaneous regression of the tumor is very rare and may occur due to unknown mechanism.(occlusion of the central retinal artery, severe inflammatory reaction and massive necrosis leading to pthisis bulbi)
Retinoblastoma is a rapidly growing tumor. If left untreated, the tumor fills the eye and completely destroys the globe in six months. Metastatic spread begins after six months and metastasized tumor is very rare at presentation. The tumor may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system or hematogenously to the lung, bone, or brain or by lymphatics if the tumor spreads anteriorly into the conjunctivae and eyelids, or extends into extraocular tissue. The most common routes of metastatic spread are direct infiltration via the optic nerve to the central nervous system, or spread via the choroid to the orbit
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:
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 |
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%)[4] Trilateral retinoblastoma has a poor prognosis, especially when tumor spreads to the sub-arachnoid space.[5] After a pineal or sellar mass is detected, the mean length of survival is 9.7 months in patients who undergo treatment and 1.3 months in those who do not.[6]
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.
- ↑ de Souza Filho JP, Martins MC, Torres VL, Dias AB, Lowen MS, Pires LA, Erwenne CM (2005). "[Histopathologic findings in retinoblastoma]". Arquivos Brasileiros De Oftalmologia (in Portuguese). 68 (3): 327–31. doi:/S0004-27492005000300010 Check
|doi=value (help). PMID 16059563. Retrieved 2012-05-07. - ↑ http://seer.cancer.gov/publications/childhood/retinoblastoma.pdf
- ↑ Provenzale JM, Gururangan S, Klintworth G (2004). "Trilateral retinoblastoma: clinical and radiologic progression". AJR. American Journal of Roentgenology. 183 (2): 505–11. PMID 15269048. Retrieved 2012-05-02. Unknown parameter
|month=ignored (help) - ↑ Holladay DA, Holladay A, Montebello JF, Redmond KP (1991). "Clinical presentation, treatment, and outcome of trilateral retinoblastoma". Cancer. 67 (3): 710–5. PMID 1985763. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help)