Maitra A, Ramnani D M, Margraf L R, Gazdar A F
Department of Pathology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75235, USA.
Pediatr Dev Pathol. 2000 Sep-Oct;3(5):492-6. doi: 10.1007/s100240010096.
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 10,000 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 4-year-old 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.
纤维板层型肝细胞癌（FHCC）是肝细胞癌的一种独特组织学变体，与经典肝细胞癌相比，它发生于更年轻的患者亚组，且预后较好。肾母细胞瘤（WT）是一种肾脏恶性胚胎性肿瘤，是儿童最常见的实体瘤之一，估计发病率为每8000至10000例出生中有1例。虽然已有关于肾母细胞瘤治疗后肝脏发生第二原发性恶性肿瘤（SMN）的报道，但肝细胞癌与肾母细胞瘤同时存在极为罕见。我们首次报告了一名此前健康的4岁女孩同时发生间变性肾母细胞瘤和纤维板层型肝细胞癌。尽管肾母细胞瘤中存在p53的局灶性免疫组化阳性，但分子分析未能发现种系或体细胞p53突变，也无法确定这两种肿瘤之间的克隆关系。
