Primary hepatic carcinoid and neuroendocrine carcinoma: Clinicopathological and immunohistochemical study of five cases

Abstract

Primary hepatic carcinoid and neuroendocrine carcinoma (NEC) are rare tumors. We experienced three carcinoids and two NEC originating in the liver during the past 25 years and attempted to elucidate the clinicopathological and immunohistochemical features of these tumors. The patients had no endocrine symptoms despite two of them having elevated plasma serotonin. Three of the five patients died of the tumor after operation with an average survival time of 20.6 months. All tumors were large (up to 26 cm in diameter), four of them solitary and one multinodular, and were not associated with liver cirrhosis. The carcinoid tumors showed insular, trabecular or glandular arrangement of argyrophilic cells, whereas in the NEC this histological pattern was distorted. Immunohistochemically the tumors showed expression of chromogranin A (all cases), chromogranin B (three cases), pancreastatin and chromostatin (four cases, respectively), prohormone convertase PC3 (three cases), carcinoembryonic antigen (CEA) and CA19-9 (two cases), cytokeratin 56 kDa (three cases), 160 kDa neurofilament (two cases) and neuron-specific enolase (two cases). Serotonin and glucagon were sporadically detected in two tumors. The most useful marker to confirm the diagnosis was chromogranin A, which was cleaved to pancreastatin and chromostatin in the tumor tissue, and was more reliable than other markers of neuroendocrine differentiation.

Computer Tomography and Magnetic Resonance Image Manifestations of Primary Hepatic Neuroendocrine Cell Carcinomas

“…Less domestic and foreign reports on PHNEC was available, as well as little understanding of its origin (Pilichowska et al, 1999;Solcia et al, 2000;Yao et al, 2008), three assumptions were proposed currently: 1) tumor cells derived from intrahepatic biliary epithelial neuroendocrine cells. 2) tumor tissue derived from the liver ectopic pancreas or adrenal tissues.…”

“…(Pilichowska et al, 1999;Modlin et al, 2008;Yao et al, 2008;Zeng et al, 2013). Liver neuroendocrine carcinoma found in clinical mostly induced by the intestines and pancreas transfer, PHNEC was very rare originating in the liver (Pilichowska et al, 1999;Tohyama et al, 2005;Hamanaka et al, 2012;Yalav et al, 2012), accounting for 1%-5% of all liver cancer, and 0.8% -4.0% of systemic neuroendocrine tumors, the imaging findings were lack of specificity, leading to difficultly preoperative diagnosis (Pilichowska et al, 1999;Bader et al, 2001;Tohyama et al, 2005;Ulusan et al, 2005;Krohn et al, 2011;Yalav et al, 2012;Baek et al, 2013). Patients generally did not have carcinoid syndrome and were lack of specific clinical symptoms and signs.…”

“…Patients generally did not have carcinoid syndrome and were lack of specific clinical symptoms and signs. They may have no symptoms when early tumor was small, they had symptoms such as upper abdominal discomfort, loss of appetite, fatigue and weight loss when the tumor increased, so the tumors often were larger when being diagnosed (Pilichowska et al, 1999;Tohyama et al, 2005;Ulusan et al, 2005). In the 19 cases of this group, 6 cases showed right hypochondrium pain discomfort, 5 cases with no obvious physical symptoms including 3 cases of weight loss, 2 cases of vomiting, 1 case of diarrhea and 1 case of stomach ulcer accidentally found by B ultrosounic inspection, one case went for treatment due to gastric ulcer and one case due to diarrhea.…”

“…The clinical hepatic neuroendocrine tumors mostly was induced by the transfer of intestines and pancreas and others (Pilichowska et al, 1999;Solcia et al, 2000;Modlin et al, 2008;Schwartz et al, 2008;Yao et al, 2008;Zeng et al, 2013) with the histopathologic performance of organ-like growth pattern, the tumor cells was argyrophilic, so they had specific positive performance in the detection of chromogranin A (CgA) and synaptophysin (Syn) of immunohistochemistry (Chang et al, 2007;Huang et al, 2010;Kim et al, 2011;Park et al, 2012). Primarily hepatic neuroendocrine carcinoma (PHNEC) was very rare, accounting for 1-5% of primary liver tumors, and 0.8%-4.0% of systemic neuroendocrine tumors (Pilichowska et al, 1999;Solcia et al, 2000;Yao et al, 2008;Huang et al, 2010). Both the clinical symptoms and imaging findings were not specific, resulting in difficult for preoperative diagnosis.…”

“…Both the clinical symptoms and imaging findings were not specific, resulting in difficult for preoperative diagnosis. Most previous studies reported in the literature were about individual case (Pilichowska et al, 1999;Tohyama et al, 2005;Yalav et al, 2010;Hamanaka et al, 2012), no reports were about the features of CT and MRI imaging for bulk cases with PHNEC (Bader et al, 2001;Ulusan et al, 2005;Rufini et al, 2006;Krohn et al, 2011;Baek et al, 2013;Li Department of Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China *For correspondence: jianqunyucn@163.com …”