Case History (Gross cont.)

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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.

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• 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.

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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.

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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
  (trilateralretinoblastoma),which is associated
  with a dismal outcome.

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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.

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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 .

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Well differentiated tumorFlexner-Wintersteiner
rosettes
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Undifferentiated tumor
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Case 7
10
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.

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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.

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