Primary Hepatic Neuroendocrine Carcinoma Coexisting with Hepatocellular Carcinoma in Hepatitis C Liver Cirrhosis: Report of a Case

Abstract

Primary hepatic neuroendocrine carcinoma is an extremely rare tumor of the liver. We herein describe a case of primary hepatic neuroendocrine carcinoma with lymph node metastases, coexisting with hepatocellular carcinoma, on a background of hepatitis C cirrrhosis, in a 72-year-old man. Abdominal ultrasonography and computed tomography (CT) showed a tumor (3 cm in diameter) in Couinaud's hepatic segment 8 (S8) with regional lymph node metastases. Whole-body CT, magnetic resonance imaging (MRI), and endoscopy did not reveal primary lesions outside the liver. Feridex MRI and [(18)F]fluorodeoxyglucose positron emission tomography were strongly suspicious of malignancy. A limited hepatectomy with regional lymph node dissection was performed. Histopathology, immunohistochemistry, and electron microscopy confirmed a diagnosis of primary neuroendocrine carcinoma on a background of liver cirrhosis. A tumor (1.5 cm in diameter) found in hepatic S5 at operation was also simultaneously resected, and histologically diagnosed to be hepatocellular carcinoma. We also review previous reports of hepatic neuroendocrine carcinoma and discuss hypotheses for the histogenesis of these tumors as well as prognostic implications. Given the background cirrhosis and coexisting hepatocellular carcinoma, we speculate that one of the hepatocellular carcinomas underwent neuroendocrine differentiation.

Tumor of the liver (hepatocellular and high grade neuroendocrine carcinoma): a case report and review of the literature

“…There are a few reports of primary neuroendocrine tumors in the liver combined with hepatocellular carcinoma, but these represented differentiation of the malignant liver cells into a neuroendocrine tumor [1,15]. The only case that appears to fulfill the criteria for collision tumor is the study reported by Ishida et al [4]. Their patient was a 72-year-old man with a 3-cm high-grade neuroendocrine carcinoma in segment 8 and a 1.5-cm moderately differentiated hepatocellular carcinoma in segment 5.…”

“…Clinically, our case had an aggressive clinical behavior during a 16-month follow-up period. The other patients with solitary high-grade neuroendocrine tumors described died within a few months after diagnosis [4]. More cases of collision and combined tumors of this type need to be documented to obtain a better insight on their pathogenesis, behavior, and treatment.…”

“…Most of the collision tumors also show a hepatocellular carcinoma accompanied by a cholangiocarcinoma. Single case reports of primary collision tumors include hepatocellular carcinoma with sarcoma [7] and the rare occurrence of hepatocellular carcinoma with neuroendocrine tumor [4], such as the one described in this article.…”

MRI findings of uncommon non-hepatocyte origin primary liver tumours with pathological correlation

“…Although the pathogenesis of primary hepatic NET has not been established, one significant hypothesis suggests that tumour cells might originate from the neuroendocrine cells in the epithelium of the intrahepatic bile duct [4,5]. These tumours are usually non-functioning neoplasms.…”

Primary neuroendocrine carcinoma coexisting with hemangioma in the liver: Report of a case

“…1 Primary neuroendocrine carcinoma (NEC) in the liver is extremely rare, and there are only 15 reports of the clinical and pathologic features of primary NEC in the English literature (Table 1). [2][3][4][5][6][7][8][9][10][11][12] The liver is a common metastatic site of NEC, unlike other sites such as the gastrointestinal tract and the pancreas. Therefore, other sites should be examined when NEC is suspected in the liver.…”