Malignant peritoneal mesothelioma in a patient with Li-Fraumeni syndrome

A 60-year-old Caucasian woman who was known to carry a germline c.473GA (p.Arg158His) mutation in the TP53 gene, which causes Li-Fraumeni syndrome (LFS), was scheduled for surgery for an umbilical hernia in another hospital. The patient’s history included a cholecystectomy for symptomatic lithiasis 20 years earlier; there was no identifiable exposure to asbestos. During surgery, pathologic thickening of the peritoneum was noted along with small soft, pinkish nodules on the visceral and parietal peritoneal surfaces. Biopsies of these lesions demonstrated a malignant peritoneal mesothelioma of the epithelioid type. Additional staging was performed using positron emission tomography– computed tomography scanning, which showed extensive [18F]fluorodeoxyglucose-avid lesions in the greater omentum and pelvis (Figs 1A and 1B) and a small amount of ascites, but no extra-abdominal disease. [...]