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.
Feb 12, 2016
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.