Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Hum Pathol. 2021 Oct;116:63-72. doi: 10.1016/j.humpath.2021.07.007. Epub 2021 Jul 24.
Primary hepatic neoplasms with neuroendocrine differentiation are extremely rare. Their clinicopathological features and molecular genetic basis are largely unknown. We identified four cases of primary hepatic neoplasms with neuroendocrine differentiation. Electronic medical records were reviewed for clinical history, imaging findings, laboratory results, and follow-up. Pathology slides, immunohistochemistry, and ancillary studies were reviewed. There were two females and two males with age ranging from 52 to 74 years. There was one amphicrine carcinoma with tumor cells simultaneously demonstrating both hepatocellular and neuroendocrine differentiation, one mixed hepatocellular-neuroendocrine carcinoma (NEC) with hepatocellular component intermingled with neuroendocrine component, one small cell NEC, and one well-differentiated neuroendocrine tumor. Next- generation sequencing of the mixed hepatocellular-NEC and small cell NEC showed molecular/genetic alterations commonly seen in hepatocellular carcinoma (HCC). All four cases arose in a background of cirrhosis. Primary hepatic neoplasms arising in cirrhotic livers can have a spectrum of neuroendocrine differentiation. Presence of a NEC component may be an indicator of aggressiveness. In addition, primary hepatic carcinomas with neuroendocrine differentiation likely share the same molecular pathways as HCC.
具有神经内分泌分化的原发性肝肿瘤极为罕见。其临床病理特征和分子遗传学基础在很大程度上尚不清楚。我们鉴定了 4 例具有神经内分泌分化的原发性肝肿瘤。回顾了电子病历中的临床病史、影像学发现、实验室结果和随访情况。回顾了病理切片、免疫组织化学和辅助研究。有 2 名女性和 2 名男性，年龄 52 至 74 岁。有 1 例 amphicrine 癌，肿瘤细胞同时具有肝细胞和神经内分泌分化，1 例混合性肝细胞-神经内分泌癌（NEC），肝细胞成分与神经内分泌成分混合，1 例小细胞 NEC 和 1 例分化良好的神经内分泌肿瘤。混合性肝细胞-NEC 和小细胞 NEC 的下一代测序显示了常见于肝细胞癌（HCC）的分子/遗传改变。这 4 例均发生在肝硬化的背景下。发生在肝硬化肝脏中的原发性肝肿瘤可以具有一系列神经内分泌分化。NEC 成分的存在可能是侵袭性的指标。此外，具有神经内分泌分化的原发性肝癌可能与 HCC 具有相同的分子途径。
