Multiple synchronous lung cancers and atypical adenomatous hyperplasia in Li-Fraumeni syndrome
Multiple synchronous lung cancers and atypical adenomatous hyperplasia in Li-Fraumeni syndrome
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DOI：
10.1046/j.1365-2559.1998.00439.x
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发表时间：
1998-07-01
期刊：
影响因子：
6.4
通讯作者：
Nicholson, AG
中科院分区：
文献类型：
--
作者：
Nadav, Y;Pastorino, U;Nicholson, AG
目的：报道一例伴有不典型腺瘤性增生的Li-Fraumeni综合征合并多个同步原发肺癌的病例，并对其发病机制进行了讨论。组织学检查发现三个独立的高分化腺癌和一个非典型腺瘤性增生的病灶，在该患者和她的其他家庭成员中都发现了P53基因的结构性突变。结论：多个同步原发肺肿瘤以前没有与这种综合征相关的报道。已知吸烟和Li-Fraumeni综合征与该患者的P53突变有关。
Aim: We report a patient with Li-Fraumeni syndrome who developed multiple synchronous primary lung cancers together with atypical adenomatous hyperplasia, and discuss the pathogenesis of the lesions,Case details: A 57-year-old woman with Li-Fraumeni syndrome and a history of smoking was diagnosed as having multiple synchronous primary lung tumours, all of which were treated by surgical excision. Histological examination showed three separate well-differentiated adenocarcinomas and a single focus of atypical adenomatous hyperplasia, A constitutional mutation of the p53 gene had been identified, both in this patient and other members of her family.Conclusion: Multiple synchronous primary lung tumours have not been reported before in association with this syndrome. With both smoking and Li-Fraumeni syndrome known to be associated with mutations in the p53 suggested in this patient.