Medical expert of the article
New publications
Diagnosis of retinoblastoma
Last reviewed: 06.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
The diagnosis of retinoblastoma is established on the basis of clinical ophthalmological examination, radiography and ultrasonography without pathomorphological confirmation. If there is a family history, children need to be examined by an ophthalmologist immediately after birth.
To confirm the diagnosis and determine the extent of the lesion (including detection of a tumor in the pineal region), CT or MRI of the orbit is recommended.
Differential diagnosis of retinoblastoma includes retinopathy of prematurity, persistent primary hyperplastic vitreous, and severe uveitis due to toxocariasis or toxoplasmosis.
Histological structure of retinoblastoma
Histologically, retinoblastoma is a monomorphic mass of small cells with a high nuclear-to-cytoplasmic ratio. Fields of small cells with blue cytoplasm are combined with pronounced vascularization. The cells tend to form the well-known "donat-like" structures formed around the clear space (these structures are also called Flexner-Wintersteiner rosettes or true retinoblastoma rosettes). The second type of typical retinoblastoma structures are florettes, resembling bouquets of flowers. Some patients with retinoblastoma have Wright rosettes - cell clusters typical of tumors of the PNET family. In extensive tumors, the necrotic component often predominates, calcifications are found in 95% of cases. The second rare subtype of retinoblastoma is represented by a thin layer of "tumor" extensively affecting the retina.
Stages of retinoblastoma
There are several staging systems for retinoblastoma. Generally, it is sufficient to subdivide the stages based on the position and size of the intraocular tumor, optic nerve involvement, orbital extension, and presence of distant metastases.
- Stage I - retinal tumor.
- Stage II - tumor of the eyeball.
- Stage III - regional extraocular spread.
- Stage IV - presence of distant metastases.
[