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dc.contributor.authorHowell, Sacha J
dc.contributor.authorHockenhull, Kimberley
dc.contributor.authorSalih, Zena
dc.contributor.authorEvans, D G
dc.date.accessioned2017-09-22T15:54:06Z
dc.date.available2017-09-22T15:54:06Z
dc.date.issued2017
dc.identifier.citationIncreased risk of breast cancer in neurofibromatosis type 1: current insights. 2017, 9:531-536 Breast Canceren
dc.identifier.issn1179-1314
dc.identifier.pmid28860858
dc.identifier.doi10.2147/BCTT.S111397
dc.identifier.urihttp://hdl.handle.net/10541/620580
dc.description.abstractNeurofibromatosis type 1 (NF1) is an autosomal dominant condition caused by mutation/deletion of the NF1 gene. The gene product, neurofibromin, is a tumor suppressor which represses the activity of the Ras oncogene. Central nervous system (CNS) tumors have long been associated with NF1, but their association with several other malignancies has been demonstrated. In this review, we summarize the epidemiological data that irrefutably support a link between NF1 and an increased risk of early-onset breast cancer, to levels at which annual mammography is currently recommended in national high-risk screening programs. We discuss the reasons for the observed adverse breast cancer prognosis in NF1 cases, including late presentation and more aggressive tumor subtypes, and recommend that a collaborative breast screening study be initiated to better serve this currently underserved population of women.
dc.language.isoenen
dc.rightsArchived with thanks to Breast cancer (Dove Medical Press)en
dc.titleIncreased risk of breast cancer in neurofibromatosis type 1: current insights.en
dc.typeArticleen
dc.contributor.departmentDepartment of Medical Oncology, The Christie NHS Foundation Trusten
dc.identifier.journalBreast Canceren
refterms.dateFOA2018-12-17T15:03:49Z
html.description.abstractNeurofibromatosis type 1 (NF1) is an autosomal dominant condition caused by mutation/deletion of the NF1 gene. The gene product, neurofibromin, is a tumor suppressor which represses the activity of the Ras oncogene. Central nervous system (CNS) tumors have long been associated with NF1, but their association with several other malignancies has been demonstrated. In this review, we summarize the epidemiological data that irrefutably support a link between NF1 and an increased risk of early-onset breast cancer, to levels at which annual mammography is currently recommended in national high-risk screening programs. We discuss the reasons for the observed adverse breast cancer prognosis in NF1 cases, including late presentation and more aggressive tumor subtypes, and recommend that a collaborative breast screening study be initiated to better serve this currently underserved population of women.


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