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AIM:The aims of this study were to identify theclinicopathological features of Chinese HNPCC families andto evaluate the value of criteria for suspected HNPCC(sHNPCC)in clinical diagnosis.METHODS:According to the follow-up records,54 HNPCCfamilies(including 12 ICG-HNPCC families and 42 sHNPCCfamilies)were screened out from patients with colorectal cancers(CRCs),operated upon in 2nd Affiliated Hospital of ZhejiangUniversity from 1984 to 2001.Clinical data of probands andtumor spectrum in these families were listed and analyzed.RESULTS:(1)Mean age,proportion of colonic cancer,poorlydifferentiated cancer,multiple CRCs and Dukes’ A+B of theprobands in ICG-HNPCC and sHNPCC kindred were 39ys and47.5ys,75 % and 62 %,0 and 12.8 %,16.7 % and 14.3 %,58.3 % and 81%,respectively.Compared with sporadiccolorectal cancers,probands from ICG-HNPCC and sHNPCCfamilies were obviously different at age of onset(P=0.025and 0.031),tumor location(P=0.001 and 0.000),differentiation(P=0.002 and 0.011)and development ofmultiple tumors(P=0.014 and 0.002).(2)A total of 178malignant neoplasms were found in 54 HNPCC families,including 139 colorectal cancers.Besides of colorectal cancer,extracolonic tumors occurred in stomach,endometrium,hepatobiliary system,and so on(8 gastric cancers,6 endometrialcancers,6 hepatobiliary system cancers and 19 others)canalso be seen in Chinese ICG-HNPCC and sHNPCC families.CONCLUSION: (1) Chinese HNPCC families have specific clinicopathological features, such as early onset, predilection for the involvement of colon, tendency of multiple CRCs, development of extracolonic tumors and well differentiation. (2) The criteria for suspected HNPCC is useful in clinical diagnosis and management of HNPCC.
AIM: The aims of this study were to identify the clinical features of Chinese HNPCC families and to evaluate the value of criteria for suspected HNPCC (sHNPCC) in clinical diagnosis. METHODS: According to the follow-up records, 54 HNPCC families (including 12 ICG-HNPCC families and 42 sHNPCCfamilies were screened out from patients with colorectal cancers (CRCs), operated upon in 2nd Affiliated Hospital of Zhejiang University from 1984 to 2001. Clinical data of probands and tumor spectrum in these families were listed and analyzed .RESULTS: (1) Mean age, proportion of colonic cancer, poorly differentiated cancer, multiple CRCs and Dukes’ A + B of the probands in ICG-HNPCC and sHNPCC kindred were 39 and 47.5ys, 75% and 62%, 0 and 12.8%, 16.7% and 14.3% 58.3% and 81%, respectively. Compared with sporadiccolorectal cancers, probands from ICG-HNPCC and sHNPCCfamilies were obviously different at age of onset (P = 0.025 and 0.031), tumor location (P = 0.001 and 0.000) and 0.011) and develop (2) A total of 178 normalignant neoplasms were found in 54 HNPCC families, including 139 colorectal cancers. Patients of colorectal cancer, extracolonic tumors occurred in stomach, endometrium, hepatobiliary system, and so on (1) Chinese HNPCC families have specific clinicopathological features, such as early onset, predilection of the involvement of the involvement of the Chinese Society of Histopathology (8 gastric cancers, 6 endometrialcancers, 6 hepatobiliary system cancers and 19 others) canalso be seen in Chinese ICG-HNPCC and sHNPCC families.CONCLUSION: of colon, tendency of multiple CRCs, development of extracolonic tumors and well differentiation. (2) The criteria for suspected HNPCC is useful in clinical diagnosis and management of HNPCC.