Retinoblastoma - advanced

Case contributed by Dr Ian Bickle

Presentation

Infant with a left sided proptosis. Parents also concerns over child's vision. Unsure of any history of trauma.

Patient Data

Age: 1
Gender: Female
CT

Left sided hyphaema.  Tiny fleck of calcification on the lateral aspect of the left globe.

CT

Patient lost to follow up and parents reluctant for medical intervention

Heavily calcified mildly enhancing mass in occupying the left globe extending into an enlarged left optic nerve, through the orbital apex into the chiasm.

This is contiguous with a large mass in the floor of the 3rd ventricle, which is causing a moderate biventricular hydrocephalus.

MRI

EVD is in situ with its tip in the region of (R) anterior frotnal lobe. Mild hydrocephalus.

1.8 x 1.2 cm calcific mass occupying most of the posterior chamber of the left eye globe.  Intraorbital and intracranial extension of the tumor along the optic nerve and optic chiasma with a resultant left proptosis.

The retrobulbar portion of the tumor measures 2.9 x 1.6 cm with widening of the optic canal. 

3.6 x 3.7 x 3.5cm mass in the suprasellar region, contiguous with the optic chiasma and encasing the A1 portion of bilateral ACA and supraclinoid portions of left ICA. This mass is compressing the floor of the third ventricle, resulting in mild hydrocephalus.

Normal right globe.

Mild thickening and enhancement of the meninges along the medial aspect of the (L) middle cranial fossa, suspicious of early leptomeningeal spread.

Case Discussion

Retinoblastoma is the commonest ocular malignancy in childhood, occuring chiefly in those under 4 years of age.  This is a case of a retinoblatoma with intraorbital and intracranial extension along the optic nerve and optic chiasma with a contiguous suprasellar mass and resultant hydrocephalus in keeping with stage IV disease.

Stage IV: metastatic disease

  • A: haematogeneous metastasis without CNS involvement
    • single lesion
    • multiple lesions or

OR

  • B: CNS metastatic involvement
    1. pre-chiasmatic lesion
    2. CNS mass
    3. leptomeningeal disease
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Case information

rID: 42598
Case created: 29th Jan 2016
Last edited: 3rd Jul 2017
Inclusion in quiz mode: Included

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