What is the efficacy of the Toronto protocol for cancer screening of patients with Li-Fraumeni syndrome?

Updated: Nov 13, 2018
  • Author: ; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Example of a surveillance program [32]

A clinical surveillance protocol (Toronto protocol) for TP53 mutation carriers was implemented at the Hospital for Sick Children in Toronto in 2004. The protocol includes frequent laboratory and radiographic screening for adrenocortical carcinoma, brain tumors, soft-tissue and bone sarcoma, leukemia/lymphoma, breast cancer, and colon cancer.

The study included 33 TP53 mutation carriers from 8 families; 18 self-elected to be in the surveillance group, and 15 self-elected to not have surveillance. Of the 18 patients in the surveillance group, 10 asymptomatic tumors were detected in 7 patients. Five tumors were malignant and 5 were low-grade or premalignant. All 7 patients were alive at the end of 24-month follow-up. Of the 16 patients in the nonsurveillance group, 12 symptomatic, high-grade, high-stage tumors were found in 10 patients. Only 2 of the 10 patients were alive at the end of follow-up.

Three-year overall survival was 100% in the surveillance group and 21% in the nonsurveillance group (P = .0155).

Among those diagnosed with a cancer, survival was better in the surveillance group than in the nonsurveillance group (100% vs 20%, respectively; P = .0417).

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