Synchronous Wilms Tumor and Fibrolamellar Hepatoce

Authors: Anirban Maitra Dharamdas M Ramnani Linda R Margraf Adi F Gazdar

Publish Date: 2014/03/01

Volume: 3, Issue: 5, Pages: 492-496

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Abstract

Fibrolamellar hepatocellular carcinoma FHCC is a unique histologic variant of HCC that occurs in a younger subset of patients than classical HCC and is associated with a better prognosis Wilms tumor WT is a malignant embryonal neoplasm of the kidney and is one of the most common solid tumors of childhood occurring at an estimated frequency of 1 in 8000 to 10000 births Although second malignant neoplasms SMNs following therapy for WTs have 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 in a previously healthy 4yearold girl Despite the presence of focal immunohistochemical positivity for p53 in the WT molecular analysis failed to reveal a germline or somatic p53 mutation and was inconclusive in establishing a clonal relation between the two tumors

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