A 3 year old baby girl presented with loss of vision.

1. What is the diagnosis?

2. Children are also at increased risk of?

3. What is this “trilateral retinoblastoma"?

Correct answer:

1. What is the diagnosis?

 Retinoblastoma

2. Children are also at increased risk of?

 Osteosarcoma

3. What is this “trilateral retinoblastoma"?

Retinoblastoma in both eyes + pinealoblastoma.


                                    Retinoblastoma

The most common intraocular neoplasm of childhood.

KEY FEATURES

- These usually occur around 18 months of age, and nearly always occur before age 5.

-There is an association with a bad RB suppressor gene, which also affects osteosarcoma patients.

-On CT think about a calcified intraocular mass, in a kid. with a normal sized globe.

- For the purpose of multiple choice tests any mass in the globe of a kid is probably RB, especially if shown on gradient (for the blooming).

- It's bilateral about 30% of the time, and can also be “trilateral” with development of a pinealoblastoma about 3 years after the ocular lesions form.

DDx:

- Coats -Not usually calcified.

- Persistent Hyperplastic Primary Vitreous (PHPV) = small globe

- Retinopathy of prematurity = small globe

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