Adrenal medullary tumors and iris proliferation in a transgenic mouse model of neurofibromatosis

J. E. Green, A. M. Baird, S. H. Hinrichs, G. K. Klintworth, G. Jay

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Abstract

The expression of the human T-cell lymphotropic virus type 1 (HTLV-1) tax gene in transgenic mice has been shown to result in the development of neurofibromas. Further characterization of these transgenic mice has revealed other significant pathologic similarities between this transgenic mouse model and human neurofibromatosis (NF). Pheochromocytomas of the adrenal medulla and hamartomas of the iris are well-recognized manifestations of human NF. Adrenal medullary tumors have been found in 68% of transgenic animals that were studied. They appear, however, not to be pheochromocytomas, but rather composed of undifferentiated spindle cells. Proliferation of fibroblastlike cells in the iris also occurs in one-half of the transgenic animals surviving more than 6 months. Melanocytes, however, have not been found in the transgenic iris lesion, although they are characteristically found in the Lisch nodules of human NF. The similarities between human neurofibromatosis and this transgenic mouse model (in which the overexpression of a single gene results in neoplasia) are discussed. This transgenic system may provide further insights into molecular mechanisms involved in the pathogenesis of neurofibromatosis.

Original languageEnglish (US)
Pages (from-to)1401-1410
Number of pages10
JournalAmerican Journal of Pathology
Volume140
Issue number6
StatePublished - Jan 1 1992

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Brain Stem Neoplasms
Neurofibromatoses
Iris
Transgenic Mice
Genetically Modified Animals
Pheochromocytoma
pX Genes
Neurofibroma
Human T-lymphotropic virus 1
Adrenal Medulla
Hamartoma
Melanocytes
Cell Proliferation
T-Lymphocytes
Genes
Neoplasms

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Adrenal medullary tumors and iris proliferation in a transgenic mouse model of neurofibromatosis. / Green, J. E.; Baird, A. M.; Hinrichs, S. H.; Klintworth, G. K.; Jay, G.

In: American Journal of Pathology, Vol. 140, No. 6, 01.01.1992, p. 1401-1410.

Research output: Contribution to journalArticle

Green, J. E. ; Baird, A. M. ; Hinrichs, S. H. ; Klintworth, G. K. ; Jay, G. / Adrenal medullary tumors and iris proliferation in a transgenic mouse model of neurofibromatosis. In: American Journal of Pathology. 1992 ; Vol. 140, No. 6. pp. 1401-1410.
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