Slide 95.Retinoblastoma, Eye

A. Brief Descriptions

  1. The most common malignant eye tumor of childhood.

  2. A cell of neuroepithelial origin in the retina.

  3. Nodular masses often with satellite seeding.

  4. Spontaneous regression: calcification and severe inflammation.

B. Gross Findings

C. Micro Findings

  1. Small round cells with large hyperchromatic nuclei and scanty cytoplasm

  2. Flexner Wintersteiner resettes:

    • Clusters of cuboidal or short columnar cells

    • Arranged around a central lumen

  3. Homer Wright rosettes:

    • Radial arrangements of cells

    • Around a central tangle of fibrils (pseudorosette)

  4. Fleurette: photoreceptor differentiation

  5. Necrosis is easily seen

D. Others

  1. Most common malignant eye tumor of childhood

  2. Characteristics:

    • Can be multifocal and bilateral

    • May undergo spontaneous regression

    • High incidence of second primary malignancy

    • Common sites of metastasis: CNS, skull, lymph nodes and distal bones

    • Frequently occurs as a congenital tumor

      • Affect both sexes equally

      • 2 patterns: familial vs. sporadic

      • Neuroepithelial origin

E. Reference

  1. Robbins pathologic basis of disease. Ch31. p.1372-1373.

  

 

 

 

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