Multiple synchronous lung cancers and atypical adenomatous hyperplasia in Li-Fraumeni syndrome.

Abstract

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. 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. 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 gene, a cumulative effect is suggested in this patient.