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伴有肠道癌的黑斑息肉综合征:一个家族中的关联报告。

Peutz-Jeghers syndrome with intestinal carcinoma: report of the association in one family.

作者信息

Hsu S D, Zaharopoulos P, May J T, Costanzi J J

出版信息

Cancer. 1979 Oct;44(4):1527-32. doi: 10.1002/1097-0142(197910)44:4<1527::aid-cncr2820440453>3.0.co;2-m.

Abstract

The Peutz-Jeghers Syndrome is a rare, genectically inherited disorder. There are insufficient data to establish this syndrome as a precancerous condition, but documented cases of gastrointestinal cancer arising in this syndrome have been reported with an incidence of 2--3%. Since the syndrome is transmitted as an autosomal dominant and is associated with cancer, family members so afflicted may show an increased cancer risk. This is the first reported case of a father and son with both Peutz-Jeghers Syndrome and colonic carcinoma.

摘要

黑斑息肉综合征是一种罕见的遗传性疾病。目前尚无足够数据将该综合征确定为癌前病变,但已有文献报道该综合征患者发生胃肠道癌症的病例,发病率为2%至3%。由于该综合征以常染色体显性方式遗传且与癌症相关,患病的家庭成员可能有更高的患癌风险。这是首例关于父子均患有黑斑息肉综合征和结肠癌的报道。

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