Retinoblastoma [Neurosurgery Wiki]

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====== Retinoblastoma ======

Retinoblastoma (Rb) is a congenital, malignant primary retinal tumor.

This rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common malignant cancer of the eye in children, and it is almost always found in young children.

40% are bilateral,90% are calcified (often a key differentiating feature; does not portend benignity as with other lesions). 

Almost all children survive this cancer, although they may lose their vision in the affected eye(s) or need to have the eye removed.

Almost half of the children with retinoblastoma have a hereditary genetic defect associated with retinoblastoma.

Sporadic retinoblastoma and [[pilocytic astrocytoma]]: A rare association of two tumors
((De Ioris MA, Carai A, Valente P, Angioni A, Randisi F, Cozza R, Romanzo A,
Marras CE, Mastronuzzi A. Sporadic retinoblastoma and pilocytic astrocytoma: A
rare association of two tumors. Pediatr Blood Cancer. 2015 Jul 14. doi:
10.1002/pbc.25636. [Epub ahead of print] PubMed PMID: 26173175.)).

===== CT =====

may show retinal detachment.

[[Coats disease]]: telangiectatic vascular malformation of [[retina]] which leaks a lipid exudate causing retinal detachment. May mimic retinoblastoma. Vitreous is hyperintense on MRI on both
T1WI and T2WI due to lipid

===== Treatment =====

[[Retinoblastoma treatment]].