Abstract

TP53 mutation, Li-Fraumeni syndrome, is a syndrome that leads to a hereditary cancer predisposition. Here we describe the case of a 13-year-old male who presented with osteosarcoma, family history of LFS, who developed a second primary tumor of the lung. No other similar cases have been reported. After this osteosarcoma diagnosis, he had pre-operative imaging which included a PET CT Chest. This revealed a subpleural nodule in the lung of unclear etiology. After completing initial therapy, a repeat chest CT showed that the nodule persisted. Pathology revealed an acinar adenocarcinoma. This tumor is not common in pediatric LFS patients.