| Hereditary tumour syndromes featuring basal cell carcinomas. | |
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MedLine Citation:
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PMID: 21428980 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Basal cell carcinoma (BCC) is the most frequent cutaneous malignancy worldwide. This skin tumour is characterised by a broad phenotypic variability and distinct histopathological subtypes. It shows slow, locally invasive growth and only rarely metastasises. BCCs can occur either sporadically or in the context of genetic syndromes, including Gorlin syndrome, Bazex-Dupré-Christol syndrome (BDCS), Rombo syndrome, Oley syndrome, and Xeroderma pigmentosum (XP). To date, different genes and signalling routes have been shown to play an important role in the development and growth of these tumours, including the hedgehog and Wnt/beta-catenin pathway. In some of the aforementioned hereditary disorders, the underlying genetic defect is still unknown whereas in others several genes have been demonstrated to be involved. Currently, most therapeutic approaches are based on surgical measures. In the case of superficial BCCs, photodynamic therapy, 5-fluorouracil cream, imiquimod or radiotherapy also may be an option. Elucidation of the molecular mechanisms governing the manifestation of BCCs in monogenetically inherited tumour syndromes will not only contribute to a better understanding of the complex pathogenesis of these tumours but might pave the way to the development of non-invasive, specific and molecule-based therapeutic strategies in the near future. |
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Authors:
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L J M T Parren; J Frank |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-3-24 |
Journal Detail:
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Title: The British journal of dermatology Volume: - ISSN: 1365-2133 ISO Abbreviation: - Publication Date: 2011 Mar |
Date Detail:
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Created Date: 2011-3-24 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0004041 Medline TA: Br J Dermatol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 British Association of Dermatologists. |
Affiliation:
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Department of Dermatology and GROW - School for Oncology and Developmental Biology; Maastricht University Medical Centre (MUMC), The Netherlands. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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