Retinoblastoma natural history, complications, and prognosis
Retinoblastoma natural history, complications, and prognosis On the Web
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If left untreated, retinoblastoma may progress to develop seeding in the eye, leading to retinal detachment, necrosis and invasion of the orbit, optic nerve invasion, and central nervous system invasion. The majority of untreated patients die of intracranial extension and disseminated disease within one year. Spontaneous regression of the tumor is a rare occurrence but may occur in a small number of cases. Common complications of retinoblastoma include metastasis, tumor recurrence, trilateral retinoblastoma, and subsequent neoplasms. Prognosis is generally good, and the survival rate of patients with retinoblastoma with treatment is approximately 95% in the United States.
Natural History, Complications, and Prognosis
- Retinoblastoma usually presents with leukocoria.
- If left untreated, retinoblastoma can be fatal. The tumor will continue growing and can invade the entire globe of the eye with subsequent metastasis.
- The tumor remains within the globe of the eye and curable within 3 to 6 months of its first presentation (when it presents with leukocoria). Delay in the diagnosis will decrease the survival rate.
- Death may occur within one year of metastasis.
- Metastasis may occur via the following four possible pathways:
- Direct invasion of the central nervous system via the optic nerve
- Through the subarachnoid space to the contralateral optic nerve
- Through the cerebrospinal fluid to the central nervous system
- To the lungs, bone, and brain via the hematogenous route
- The tumor may also spread via the lymphatics if the tumor invades anteriorly into the conjunctivae, eyelids, or extraocular tissue.
- Spontaneous regression of the tumor is a rare occurrence but may occur in a small number of cases.
- Massive choroidal invasion
- Tumor invasion into the anterior chamber
- Large tumor size with vitreous seeding
- Neovascularization of the iris
- Elevated intracranial pressure (ICP)
- Those with heritable form of the disease have 50% risk of transmitting the mutation to their offspring.
- Regarding the variable accessibility of patients to the resources, the survival rate may range from < 30% in low and middle income societies to > 90% in developed countries.
- The overall 5-year survival rate increased over the years and was reported 97.3% from 2000 to 2012.
- Prognosis is generally good, and the survival rate of patients with retinoblastoma with treatment is approximately 95%, in the United States.
- The survival rate is higher for unilateral involvement than the bilateral form of the tumor.
- It has been observed that survival rate varies depending upon the following factors:
- The overall prognosis of trilateral retinoblastoma is poor and patients usually die within the first year of the diagnosis.
- Intraocular Classification of Retinoblastoma (ICRB) has been observed to have the ability to predict the outcome of chemotherapy:
- Prognosis is usually poor with non-ocular tumor and it usually occurs in individuals who have received radiation therapy for their primary retinoblastoma tumors.
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