P-J综合征小肠息肉癌变——附一个家族两代4例病例报道及文献回顾

Carcinomatous Changes in Hamartomatous Polyps of the Peutz-Jegher′s Syndrome of Small Intestine

本文报道一个家族两代4例P-J综合征(Peutz-Jegher′s Syndrome)。母亲于43岁并发十二指肠癌。手术中因癌已与胰腺粘连未能完全切除,术后1月死亡。切除肠段病理检查在息肉与肠癌交界处可见从错构瘤表面腺管垂直延长。至单一粘液性细胞腺瘤性增生、至不典型增生、最终演变为粘液腺癌的逐级过渡图像,支持P-J综合征错构瘤性息肉可以癌变的观点。

Four cases of Peutz-Jepher's(P-J)syndrome in a family of two generatians:moth-er,two daughters and one son were reported.The mother camplicated carcinoma in her duode-num at 43 age,On histological examination ofthe resected duodenum,the authors had re-searched those worthy features as follows:at theborderline of hamartomatous polyps and cancer,the crypt of hamartoma stretched straightly atfirst;only the mucous cells became adenomatousproliferation;then followed by atypical prolifera-tion and mucous carcinoma transformation.Thereseach strongly support the view that carcino-mas may arise from hamartomatous polyps of P-J syndrome.