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与立陶宛普通人群相比,HNPCC家族中结直肠癌患者的生存率——初步结果。

Survival from colorectal carcinoma in HNPCC families as compared to the general population in Lithuania--initial results.

作者信息

Elsakov Pavel, Kurtinaitis Juozas

机构信息

Institute of Oncology, Vilnius University, Santariskiu str. 1, 2021, Vilnius, Lithuania.

出版信息

Fam Cancer. 2006;5(4):369-71. doi: 10.1007/s10689-006-0007-7. Epub 2006 Jul 7.

Abstract

BACKGROUND

Colorectal cancer in HNPCC appears to have a better prognosis than sporadic colorectal cancer. Selection bias, screening, early intervention and treatment hamper efforts to confirm such a hypothesis.

AIM

To evaluate survival rates in Lithuanian HNPCC patients with colorectal cancer and compare them with survival rates of sporadic cases arising from the general population.

PATIENTS AND METHODS

The study group consisted of 8 patients from 6 Hereditary non-polyposis colorectal carcinoma (HNPCC) families, 3 patients had HMSH2 and 5 patients had HMLH1 mutations, who were diagnosed between 1995 and 1999. HNPCC patients characteristic (age and stage) were used to trace the records of the Cancer Registry at the same period to identify the cases corresponding the required criteria. Above 263 patients were found--106 at stage II and 157 at stage III.

RESULTS

The 10-year survival was 87.5% in the HNPCC study group compared with only 44.8% in the general population group. Patients with the MSH2 gene mutation were at a greater risk of developing a second primary cancer independent from any cancer prevention and screening programs. Survival rates were also prolonged even with a greater numbers of extra-colonic cancers diagnosed at different stages.

CONCLUSION

HNPCC patients with confirmed MSH2 or MLH1 mutations diagnosed with stages II and III CRC have a good 10-year survival prognoses compared with those from the general population.

摘要

背景

遗传性非息肉病性结直肠癌(HNPCC)患者的结直肠癌预后似乎比散发性结直肠癌更好。选择偏倚、筛查、早期干预和治疗阻碍了证实这一假设的努力。

目的

评估立陶宛HNPCC结直肠癌患者的生存率,并将其与普通人群中散发性病例的生存率进行比较。

患者与方法

研究组由来自6个遗传性非息肉病性结直肠癌(HNPCC)家族的8名患者组成,其中3名患者有HMSH2基因突变,5名患者有HMLH1基因突变,这些患者于1995年至1999年期间被诊断。利用HNPCC患者的特征(年龄和分期)追溯同期癌症登记处的记录,以确定符合所需标准的病例。共找到263例以上患者——106例处于II期,157例处于III期。

结果

HNPCC研究组的10年生存率为87.5%,而普通人群组仅为44.8%。MSH2基因突变的患者发生第二原发性癌症的风险更高,且与任何癌症预防和筛查计划无关。即使在不同阶段诊断出更多的结肠外癌症,生存率也有所延长。

结论

与普通人群相比,确诊为MSH2或MLH1基因突变且处于II期和III期结直肠癌的HNPCC患者有良好的10年生存预后。

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