Case History (Gross cont.)

• Heavily pigment deposits were seen in the angle and on the surface of the blue iris.

• Lens was in place, and the vitreous was collapsed anteriorly.

• Optic nerve head was deeply cupped.

• Diagnosis– Retinoblastoma without rosettes– Extensive neoplastic invasion of the optic nerve,

meninges, and subarchnoid space to the plane of surgical section.

• Comment.– Prognosis in this case is poor because of extensive

invasion of the optic nerve and meninges.

Retinoblastoma (RB)• Most common primary intraocular malignancy

of children. • Although the name might suggest origin from

a primitive retinal cell capable of glial and neuronal differentiation, it is now clear that the cell of origin is neuronal.

• Prognosis is adversely affected by extraocular extension and invasion along the optic nerve and possibly, choroidal invasion.

Retinoblastoma (RB)

• Recall :Approximately 40% of cases are inherited through a germ-line mutation of a single Rb allele.

• Cases arising in the context of germ line mutations may be bilateral and may even be associated with pinealoblastoma (“trilateral“retinoblastoma),which is associated with a dismal outcome.

Retinoblastoma (RB)

• Pathology of both hereditary and sporadic types is identical.

• Tumor may contain both undifferentiated and differentiated elements. – Undifferentiated tumor: Collections of small,

round cells with hyperchromatic nuclei.– Well-differentiated tumors: Flexner-Wintersteiner

rosettes and fleurettes reflecting photoreceptor differentiation.

Retinoblastoma (RB)• Degree of differentiation does not appear to

be associated with prognosis. • Viable tumor cells encircle tumor blood

vessels with zones of necrosis typically found in relatively avascular areas.

• Focal zones of dystrophic calcification are characteristic .

Well differentiated tumor:Flexner-Wintersteiner rosettes

Undifferentiated tumor

Case 7

Case History

• 77 yo WM• Bilateral chronic glaucoma and cataracts • Uncontrollable Glaucoma OD• Enucleation• At the time of enucleation, the patient was

blind in both eyes.

Case History (Gross)• 23.5 x 22.5 x 22mm OD; the optic nerve was cut flash

with the globe.• Clear cornea11x10mm• Globe transmitted light evenly • Opened in the horizontal plane • Chamber angle : narrow• Lens was in place.• Extensive deep and superficial retinal hemorrhages• Optic nerve head was deeply cupped.