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Retinoblastoma: Diagnosis, Prognosis, and Pathology Overview

Explore the case history and gross examination of retinoblastoma, a common childhood intraocular malignancy. Learn about the diagnosis, prognosis, and pathology of this condition affecting the optic nerve, meninges, and subarachnoid space.

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Retinoblastoma: Diagnosis, Prognosis, and Pathology Overview

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  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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 .

  7. Well differentiated tumor:Flexner-Wintersteiner rosettes

  8. Undifferentiated tumor

  9. 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.

  11. 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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