Osteosarcomatosis with rothmund-thomson syndrome
We describe the second reported case of multicentric osteosarcoma associated with Rothmund-Thomson syndrome (RTS), a rare hereditary cancer-prone genodermatosis characterized by typical cutaneous lesions and other non-dermatological pathological manifestations, particularly skeletal changes. A high...
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| Format: | article |
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1977
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| Online Access: | http://hdl.handle.net/10725/10478 https://doi.org/10.1259/bjr.70.830.9135453 http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php https://www.birpublications.org/doi/abs/10.1259/bjr.70.830.9135453 |
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| Summary: | We describe the second reported case of multicentric osteosarcoma associated with Rothmund-Thomson syndrome (RTS), a rare hereditary cancer-prone genodermatosis characterized by typical cutaneous lesions and other non-dermatological pathological manifestations, particularly skeletal changes. A high incidence of malignant disorders has been found in RTS patients. This could be explained by the diminished capacity of DNA-repair demonstrated in the fibroblasts of RTS patients following exposure to oncogenic stimuli. The severe cutaneous lesions and the benign bone lesions found in RTS may be considered a predisposing factor to the particularly frequent skin carcinoma and osteogenic sarcoma encountered in these cancer-prone patients. A brief review of the literature is given, with a discussion of the association between these two rare conditions. |
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