Cantonal Institute of Pathology, Liestal, Switzerland.
Diagn Pathol. 2011 Aug 10;6:75. doi: 10.1186/1746-1596-6-75.
Ectopic liver is a very uncommon developmental anomaly that predisposes to the development of hepatocellular carcinoma. We describe the second documented case of a hepatocellular carcinoma developing in the primary liver of a patient with a rare and uncharacterized genetic symptom complex. Also present was the largest ectopic liver ever reported, measuring 12 cm in diameter which contained a solitary focus of metastatic hepatocellular carcinoma. The primary hepatocellular carcinoma is believed to have arisen in the native liver from a hepatic adenoma that was diagnosed 15 years earlier. The patient's uncharacterised condition featured prominent thick, yellow skin over the dorsum of the fingers, and was associated with follicular hyperkeratosis, abnormal plantar creases, digital clubbing, misshaped ears, a lingua plicata and an angioleiomyolipoma of the right kidney. This unique case of hepatocellular carcinoma arising from liver cell adenoma in a patient with an uncharacterised condition featuring a large ectopic liver invites discussion of the role of local factors in carcinogenesis in the parent liver but not the ectopic liver. It also underlines the imperative ongoing need for clinical autopsies.
异位肝是一种非常罕见的发育异常，易导致肝细胞癌的发生。我们描述了第二例罕见且特征不明的遗传症状复杂患者的原发性肝脏中发生肝细胞癌的病例。还存在着迄今为止报道的最大的异位肝，直径为 12 厘米，其中包含一个孤立的转移性肝细胞癌病灶。原发性肝细胞癌被认为是从 15 年前诊断出的肝腺瘤在原肝中发生的。该患者的特征不明的病症表现为手指背部明显的厚而黄的皮肤，伴有毛囊角化过度、足底异常折痕、杵状指、耳朵畸形、舌系带过短和右肾血管平滑肌脂肪瘤。这种源于肝母细胞瘤的肝细胞癌的独特病例，发生在一个具有大的异位肝的特征不明的病症患者中，提示局部因素在亲代肝脏而非异位肝脏中的致癌作用。它还强调了临床尸检持续必要性。
