[Unexpected liver tumors in a patient with Li-Fraumeni syndrome]

Liver involvement in Li-Fraumeni syndrome is usually due to primary or metastatic malignant tumors. We report a case in which multiple liver nodules proved to be benign lesions. This 30-year-old female patient, with a known Li-Fraumeni syndrome and a history of several previous malignancies, presented multiple liver nodules detected at systematic annual screening. Two were suggestive of focal nodular hyperplasia. Three others were fat-containing at imaging studies; a guided biopsy of the largest lesion, measuring 15mm in diameter, concluded to liver angiomyolipoma, of mixed composition, combining adipocyte-like cells, dystrophic vascular structures, spindle cells and epithelioid cells coexpressing smooth muscle and melanocytic markers. There was no adverse histopathological feature. A simple surveillance was decided. The possible occurrence of benign lesions during Li-Fraumeni syndrome, requiring only conservative management after histopathological diagnosis, must be known, especially with the increasing use of annual whole-body imaging for screening.