Tumor development mechanism and its risk evaluation in Werner syndrome

点击翻译按钮获取中文摘要

Werner syndrome is an interesting model for researches both of aging and cancer. We have so far revealed that six kinds of rare tumors, i.e. thyroid carcinoma, bone sarcoma, soft part sarcoma, myeloid disorders/MDS, meningioma and melanoma, were increased and that tumors common in general populations were not increased in Werner syndrome.In the current study, we have investigated (1) relationships between WRN helicase deficit and tumorigenesis and (2) evaluation of tumor risk in Werner syndrome.Regarding the first issue, for further analysis in the future, we collected seven cases of lung malignant tumors because relations between tumor causes and histology/genetic changes were relatively well established in lung tumors.For the second issue, we gathered data and/or materials of 133 non-epithelial (including 110 malignant) and 75 epithelial tumors from our related institutions and research collaborators or through examining public data bases such as the PubMed and the Igaku-Chuo-Zasshi. As a result, we updated our tumor files, providing the new tumor spectrum : 37 soft part sarcoma (including subcutaneous ones), 34 melanomas, 23 leukemia/MDS, 21 meningiomas, 14 bone sarcomas, 4 brain tumors as well as 28 thyroid carcinomas, 10 skin carcinomas, 7 liver carcinomas, 6 breast carcinomas, 6 gastric carcinomas, 5 lung carcinomas, 1 colonic carcinoma and no prostatic carcinoma. As compared with the previous statistics published in 1996, increased soft part sarcoma and skin cancers other than melanoma were noted. Particularly, it was interesting that we had now as many as 39 malignant tumors related to skin, including subcutaneous ones. Details were 23 melanomas, 5 basal cell carcinomas, 3 squamous cell carcinomas, 1 sebaceous gland carcinoma, 1 Bowen's disease, and 1 carcinoma NOS. Benign skin tumors were 1 solar keratosis, 1 nevocellular nevus, and subcutaneous sarcomas included 2 malignant fbrous histioctomas and 1 acute T cell leukemia.