Li-Fraumeni-Syndrom

Synchronous

choroid plexus papilloma and Wilms tumor in a girl, disclosing a Li-Fraumeni syndrome. Radiological studies. Panel a Intravenous contrast-enhanced abdominal CT, coronal view: a solid, heterogeneous tumor in the upper pole of the left kidney is identified (upper, thin arrow), with an adjacent subcapsular perirenal hematoma (lower, thick arrow). The actual diameters were 36 mm × 28 mm × 27 mm in anteroposterior, transverse, and craniocaudal planes. Panel b Cerebral MRI, T2-weighted sequence, axial view. A solid supratentorial intraventricular tumor with left parietal lobe extension is shown. Cystic areas in the basal peripheral and medial aspects are identified .The tumor causes localized ventriculomegaly, vasogenic edema and midline shift. The actual diameters (tumor and cysts) were 81 × 59 × 75 mm in the anteroposterior, transverse, and craniocaudal planes. Panel c Non-enhanced Brain CT scan after shunt insertion. Axial reconstruction. A left parietal tumor with extensive calcification is shown. Ventricular catheter tip is in the right frontal ventricular horn