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.