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Diagnosis of retinoblastoma
Last reviewed: 23.04.2024
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The diagnosis of retinoblastoma is established on the basis of clinical ophthalmological examination, radiography and ultrasonography without pathomorphological confirmation. In the presence of a family history, children should be examined by an ophthalmologist immediately after birth.
To confirm the diagnosis and determine the extent of the lesion (including detection of the tumor of the pineal region) CT or MRI of the orbit is recommended.
Differential diagnosis of retinoblastoma is carried out with retinopathy of prematurity, persistent primary hyperplastic vitreous body and severe uveitis due to toxocarosis or toxoplasmosis.
Histological structure of retinoblastoma
Histologically, retinoblastoma is represented by a monomorphic mass of small cells with a high nuclear-cytoplasmic ratio. The fields of small cells with blue cytoplasm are combined with pronounced vascularization. Cells tend to form well-known "donat-like" structures formed around a light space (these structures are also called Flexner-Winterstein outlets or true retinoblastoma rosettes). The second type of typical retinoblastoma structures is a flower pattern resembling a bouquet of flowers. In some patients retinoblastoma is detected by Wright's rosettes - cell aggregations, typical for tumors of the PET family. In extensive tumors, the necrotic component often prevails, calcification is found in 95% of cases. The second rare subtype of retinoblastoma is represented by a thin layer of "tumor", which extensively affects the retina.
Stages of retinoblastoma
There are several systems for the staging of retinoblastoma. As a rule, it is sufficient to subdivide the stages based on the position and size of the intraocular tumor, the involvement of the optic nerve, the propagation in the orbit, and the presence of distant metastases.
- Stage I - a retinal tumor.
- Stage II - a tumor of the eyeball.
- Stage III - regional extraocular distribution.
- Stage IV - the presence of distant metastases.
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