Abstract:
:The most common NF1 feature is the benign neurofibroma, which consists predominantly of Schwann cells. Dermal neurofibromas usually arise during puberty and increase in number throughout adulthood. Plexiform neurofibromas, associated with larger nerves, are often congenital and can be life threatening. Malignant peripheral nerve sheath tumors (MPNST) in NF1 are believed to arise from plexiforms in 5%-10% of patients. There are reports of increased potential for malignant transformation of plexiform tumors and increase in dermal neurofibromas, during pregnancy. These observations suggest that steroid hormones influence neurofibroma growth, and our work is the first to examine steroid hormone receptor expression and ligand-mediated cell growth and survival in normal human Schwann cells and neurofibroma-derived Schwann cell cultures. Immunohistochemistry and real-time PCR showed that estrogen receptors (ERs), progesterone receptor (PR), and androgen receptor are differentially expressed in primary neurofibromas and in NF1 tumor-derived Schwann cell cultures compared to normal Schwann cells. However, there is substantial heterogeneity, with no clear divisions based on tumor type or gender. The in vitro effects of steroid hormone receptor ligands on proliferation and apoptosis of early passage NF1 tumor-derived Schwann cell cultures were compared to normal Schwann cell cultures. Some statistically significant changes in proliferation and apoptosis were found, also showing heterogeneity across groups and ligands. Overall, the changes are consistent with increased cell accumulation. Our data suggest that steroid hormones can directly influence neurofibroma initiation or progression by acting through their cognate receptor, but that these effects may only apply to a subset of tumors, in either gender.
journal_name
Mol Carcinogjournal_title
Molecular carcinogenesisauthors
Fishbein L,Zhang X,Fisher LB,Li H,Campbell-Thompson M,Yachnis A,Rubenstein A,Muir D,Wallace MRdoi
10.1002/mc.20236subject
Has Abstractpub_date
2007-07-01 00:00:00pages
512-23issue
7eissn
0899-1987issn
1098-2744journal_volume
46pub_type
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更新日期:2019-06-01 00:00:00
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pub_type: 杂志文章
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更新日期:1992-01-01 00:00:00
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更新日期:2009-06-01 00:00:00
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更新日期:1991-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2001-08-01 00:00:00
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更新日期:2020-07-01 00:00:00
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更新日期:2016-12-01 00:00:00
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更新日期:2008-05-01 00:00:00
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更新日期:2012-10-01 00:00:00
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更新日期:1994-08-01 00:00:00
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journal_title:Molecular carcinogenesis
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更新日期:1995-01-01 00:00:00
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更新日期:2014-05-01 00:00:00
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更新日期:1999-02-01 00:00:00
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更新日期:2017-01-01 00:00:00