Diffuse Cirrhosis-like Hepatocellular Carcinoma A Clinically and Radiographically Undetected Variant Mimicking Cirrhosis

Diffuse Cirrhosis-like Hepatocellular Carcinoma A Clinically and Radiographically Undetected Variant Mimicking Cirrhosis
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DOI:
10.1097/pas.0b013e3181ddf52f
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发表时间:
2010-07-01
影响因子:
5.6
通讯作者:
Ferrell, Linda D.
Ferrell, Linda D.
中科院分区:
医学1区
文献类型:
--
作者:
Jakate, Shriram;Yabes, Annoel;Ferrell, Linda D.

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肝细胞癌(HCC)的一种罕见变异是在整个肝脏中弥漫性产生小的结节样结节(CL-HCC),而不是更大的明显肿块。这种类型的肿瘤在临床上和放射学上都没有被发现,但在自体肝移植后却意外地被发现。我们研究了来自4个医疗中心的10例此类病例(9男1女,年龄35 - 80岁)。移植前的临床,实验室和放射学研究进行了审查,以确定肝脏疾病的原因和阶段,甲胎蛋白(AFP)水平,和检测的质量成像。所有10例患者均存在不同病因的基础肝硬化[3例丙型肝炎病毒(HCV),3例酒精性肝炎,1例B型肝炎病毒,1例自身免疫性,2例混合型HCV/酒精性肝炎和血色素沉着症/HCV],并接受了原位肝移植,术前临床上未怀疑HCC。超声和/或动态成像显示肝硬化,没有明确的HCC。AFP水平仅在10例病例中的3例中轻度升高(144、150和252 ng/mL)。大体上,有无数(约20至> 1000)小CL-HCC结节(0.2至0.6 cm)散布在结节之间。组织学上,这些都是高分化或中等分化的HCC,通常具有假腺型,结节周围的SCREEN边缘,胆汁淤积,频繁的马洛里体,和小血管侵犯。CL-HCC除具有常见的HCC免疫表型外,免疫组化显示频繁的泛素、胞浆和胞膜CD 10阳性,Ki-67增殖指数相对较低,AFP阴性。CL-HCC作为一种独特的HCC变异体,尽管有巨大的肿瘤负荷,但仍能逃避移植前的检测,模仿结节,并显示出一些不常见的病理和免疫表型特征。
A rare variant of hepatocellular carcinoma (HCC) is encountered that produces small cirrhosis-like nodules diffusely throughout the liver (CL-HCC), instead of a larger evident mass. This pattern remains undetected as carcinoma clinically and radiographically and is unexpectedly discovered after liver transplantation in the explanted native liver. We studied 10 such cases (9 males and 1 female, age 35 to 80 y) from 4 medical centers. The pretransplant clinical, laboratory, and radiographical studies were reviewed to determine the cause and stage of liver disease, alpha-fetoprotein (AFP) levels, and detectability of a mass on imaging. All 10 cases had underlying cirrhosis of varying etiology [3 hepatitis C virus (HCV), 3 alcoholic hepatitis, 1 hepatitis B virus, 1 autoimmune, and 2 mixed HCV/alcoholic hepatitis and hemochromatosis/HCV] and underwent orthotopic liver transplantation with no preoperative clinical suspicion of HCC. Ultrasound and/or dynamic imaging showed cirrhosis and no definite HCC. AFP levels were only mildly elevated in only 3 of 10 cases (144, 150, and 252 ng/mL). Grossly, there were innumerable (from about 20 to > 1000) small CL-HCC nodules (0.2 to 0.6 cm) scattered among cirrhotic nodules. Histologically, these were well or moderately differentiated HCC, often with pseudoglandular pattern, perinodular sclerotic rims, cholestasis, frequent Mallory bodies, and small vessel invasion. In addition to the usual HCC immunophenotype, CL-HCC showed frequent ubiquitin and cytoplasmic and membranous CD10 positivity, relatively low Ki-67 proliferative index and absence of AFP immunohistochemically. CL-HCC warrants recognition as a unique HCC variant that evades pretransplant detection despite massive tumor burden, mimics cirrhotic nodules, and shows some uncommon pathologic and immunophenotypical characteristics.