Synchronous Wilms tumor and fibrolamellar hepatocellular carcinoma: Reportof a case

Fibrolamellar hepatocellular carcinoma (FHCC) is a unique histologic varian t of HCC that occurs in a younger subset of patients than classical HCC, an d is associated with a better prognosis. Wilms tumor (WT) is a malignant em bryonal neoplasm of the kidney and is one of the most common solid tumors o f childhood, occurring at an estimated frequency of 1 in 8000 to 10,000 bir ths. Although second malignant neoplasms (SMNs) following therapy for WTs h ave been reported in the liver, the coexistence of HCC and WT is extremely rare. We present the first report of a synchronous anaplastic WT and FHCC i n a previously healthy 4-year-old girl. Despite the presence of focal immun ohistochemical positivity for p53 in the WT, molecular analysis failed to r eveal a germline or somatic p53 mutation, and was inconclusive in establish ing a clonal relation between the two tumors.