Primary Hepatic Neoplasms Arising in Cirrhotic Livers can have a Variable Spectrum of Neuroendocrine Differentiation.

Abstract

PURPOSES\nPrimary hepatic neoplasms with neuroendocrine differentiation are extremely rare. Their clinicopathological features and molecular genetic basis are largely unknown.\n\n\nMETHODS AND RESULTS\nWe 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 72 years. There was one amphicrine carcinoma with tumor cells simultaneously demonstrating both hepatocellular and neuroendocrine differentiation, one mixed hepatocellular-neuroendocrine carcinoma with hepatocellular component intermingled with neuroendocrine component, one small cell neuroendocrine carcinoma, and one well-differentiated neuroendocrine tumor. Next generation sequencing of the mixed hepatocellular-neuroendocrine carcinoma and small cell neuroendocrine carcinoma showed molecular/genetic alterations commonly seen in hepatocellular carcinoma. All four cases arose in a background of cirrhosis.\n\n\nCONCLUSIONS\nPrimary hepatic neoplasms arising in cirrhotic livers can have a spectrum of neuroendocrine differentiation. Presence of a neuroendocrine carcinoma component may be an indicator of aggressiveness. In addition, primary hepatic carcinomas with neuroendocrine differentiation likely share the same molecular pathways as hepatocellular carcinoma.