Retinoblastoma natural history, complications, and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
If left untreated, the tumor fills the eye and completely destroys the globe in six months .Retinoblastoma is a rapidly growing tumor. 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. If untreated, death usually occurs in few years.
Natural History
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
Good prognosis depends upon early presentation of the child in health facility. Late presentation of the child in hospital is associated with poor prognosis.
Survival
Children with a germline RB1 mutation may continue to develop new tumors for a few years after diagnosis and treatment; for this reason, they need to be examined frequently. It is common practice for examinations to occur every 2 to 4 months for at least 28 months. The interval between exams is based on the stability of the disease and age of the child (i.e., less frequent visits as the child ages). A proportion of children who present with unilateral retinoblastoma will eventually develop disease in the opposite eye. Periodic examinations of the unaffected eye are performed until the germline status of the RB1 gene is determined.
Complications
The complications of retinoblastoma may be
- Local spread of tumor
- Development of other tumors
- Cataract from radiotherapy
- Failure of tooth eruption after radiotherapy
- Bony deformities
References
- ↑ Singh, Arun D., Carol L. Shields, and Jerry A. Shields. "Prognostic factors in retinoblastoma." Journal of pediatric ophthalmology and strabismus 37.3 (2000): 134.
See also
Template:Nervous tissue tumors