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Osteosarcomatosis with Rothmund-Thomson syndrome el-Khoury JM; Haddad SN; Atallah NGBr J Radiol 1997[Feb]; 70 (ä): 215-8We 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.|*Tibia/diagnostic imaging[MESH]|Bone Neoplasms/*complications/diagnostic imaging[MESH]|Child[MESH]|Female[MESH]|Humans[MESH]|Knee/diagnostic imaging[MESH]|Osteosarcoma/*complications/diagnostic imaging/etiology[MESH]|Radiography[MESH]|Rothmund-Thomson Syndrome/*complications[MESH]|Syndrome[MESH] |
