Clinical characteristics and outcome of primary hepatic neuroendocrine tumors after comprehensive therapy

Abstract

BACKGROUND Primary hepatic neuroendocrine tumors (PHNETs), a group of neuroendocrine neoplasms, are extremely rare. There are only few case reports about PHNETs in the literature. The lack of large samples and multicenter research results in poor diagnostic and therapeutic approaches. AIM To discuss the clinical characteristics, diagnosis, and treatment of PHNETs and risk factors related to survival. METHODS We retrospectively analyzed the clinical data, imaging features, immunohistochemistry data, and treatment efficacy of 40 patients who were pathologically diagnosed with PHNETs and admitted to The First Affiliated Hospital of Zhengzhou University from January 1, 2014 to November 15, 2019. Finally, survival analysis was performed to identify the risk factors for survival. RESULTS The main symptoms and signs included intermittent abdominal pain (19 patients, 47.5%) and bloating (8 patients, 20.0%). The positive rates of tested tumor markers were recorded as follows: Carbohydrate antigen 19-9 (CA19-9) (6 patients, 15.0%), CA72-4 (3 patients, 7.5%), carcinoembryonic antigen (7 patients, 17.5%), and alpha-fetoprotein (6 patients, 15.0%). Immunohistochemical staining results showed positivity for Syn in 38 (97.4%) of 39 patients, for chromogranin A in 17 (65.4%) of 26 patients, for CD56 in 35 (94.6%) of 37 patients, for AE1/AE3 in 28 (87.5%) of 32 patients, and for Ki-67 in all 40 (100.0%) patients. The overall survival rate was significantly related to the tumor grade, AE1/AE3, and Ki-67. No significant correlation was found between other parameters (age, gender, tumor number, tumor size, metastasis, and treatment) and overall survival. CONCLUSION Higher grade, negative AE1/AE3, and higher Ki-67 are associated with a worse survival rate. Kinds of treatment and other parameters have no significant influence on overall survival.

Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report

“…Both CA19-9 and CA-125 are commonly used serum tumor markers for the preoperative diagnosis of cholangiocarcinoma [ 8 ]. However, Wang et al [ 9 ] reported that the positivity of CA19-9 was only 15.0%, CA72-4 was 7.5%, CEA was 17.5%, and AFP was 15.0% in primary hepatic neuroendocrine tumors. Furthermore, serum tumor markers are normal in most EBNET G1/G2, but they tend to be higher than normal levels in EBNET G3 or extrahepatic biliary neuroendocrine carcinomas [ 10 – 14 ].…”

Primary hepatic neuroendocrine tumour with multiple liver metastases: A case report with literature review

“…Studies have shown that the mitotic index and Ki-67 index are of great value in evaluating the malignancy of PHNEN and its prognosis [18] . Wang et al [19] analysed the survival of 40 PHNET patients and found that the tumour grade and Ki-67 index were significantly related to the overall survival rate. Our case showed a low cell proliferation index, such as 2% Ki-67 and less than 2 mitoses in every 10 high-power fields.…”

Efficacy of Chemotherapy versus Transcatheter Arterial Chemoembolization in Patients with Advanced Primary Hepatic Neuroendocrine Carcinoma and an Analysis of the Prognostic Factors: A Retrospective Study

“…3 , 14 , 15 TACE, platinum-based chemotherapy (EP/EC), radiofrequency ablation (RFA), and liver transplantation have been used to treat PHNEC in previous cases. 3 , 6 , 11 , 16–18 However, a systematic analysis of the treatment outcomes and prognostic factors in patients with PHNEC has not been conducted. In our study, 23 patients with advanced PHNEC received EP/EC, and 18 patients received TACE treatment.…”