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

Abstract

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 gene, a cumulative effect is suggested in this patient.