February 1991
Volume 32, Issue 2
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Articles  |   February 1991
Primitive neuroectodermal tumor of the midbrain in a murine model of retinoblastoma.
Author Affiliations
  • D M Marcus
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • J L Carpenter
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • J M O'Brien
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • T Kivela
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • E Brauner
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • A Tarkkanen
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • I Virtanen
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
  • D M Albert
    David G. Cogan Eye Pathology Laboratory, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114.
Investigative Ophthalmology & Visual Science February 1991, Vol.32, 293-301. doi:
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      D M Marcus, J L Carpenter, J M O'Brien, T Kivela, E Brauner, A Tarkkanen, I Virtanen, D M Albert; Primitive neuroectodermal tumor of the midbrain in a murine model of retinoblastoma.. Invest. Ophthalmol. Vis. Sci. 1991;32(2):293-301.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

The first heritable model of retinoblastoma was established by retina-specific expression of simian virus 40 T-antigen (SV40 T-ag) in transgenic mice. Bilateral, multifocal ocular tumors were observed in 100% of transgene-bearing mice. Central nervous system neoplasms occurred at a lower rate (27%) and represented the murine counterpart of human trilateral retinoblastoma. The authors characterized the transgenic brain tumors and found them to be primitive neuroectodermal tumors (PNET) of the midbrain. Murine brain tumors do not involve the pineal gland and most closely resemble undifferentiated suprasellar or parasellar tumors occasionally observed in human trilateral retinoblastoma. The murine malignancies arose from the subependymal cells of the cerebral aqueduct. Immunohistochemical and ultrastructural examination revealed that the transgenic brain tumors were undifferentiated and lacked all antigens associated with normal murine neuronal, glial, and ependymal cells.

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