Colon Pathology Characteristics in Li-Fraumeni Syndrome.

Specialty Areas:
Date: November 28, 2017
Authors:
Hailey M. Shepherd, Joshua D. Schiffman, Kory W. Jasperson, N. Jewel Samadder, Sheryl R. Tripp, Wade Samowitz, Wendy Kohlmann, William Rengifo-Cam
Journal: Clinical Gastroenterology and Hepatology

Li–Fraumeni syndrome (LFS) is a rare autosomal-dominant hereditary cancer syndrome associated with germline mutations in the TP53 tumor-suppressor gene. The lifetime risk of cancer is up to 70% in men and almost 100% in women.1 Studies continue to show that the tumor spectrum of TP53 mutation carriers is wider than previously thought and includes early onset colorectal cancer (CRC).2–4