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.

Keywords: Diagnosis; Neuroendocrine tumors; Primary hepatic neuroendocrine tumors; Survival analysis; Treatment; Tumor grade.

Figure 1

Survival analysis showed that tumor grade, AE1/AE3, and Ki-67 were significantly related to the overall survival rate.

Figure 2

There are multiple low-density lesions in the liver with nonuniform radioactive distribution. A and B: The size of the largest lesion is 2.3 cm × 2.8 cm, and the computed tomography value is 36 HU; C and D: Smaller lesion.

Figure 3

Comparison of computed tomography images of the liver lesion before and after long-acting repeatable octreotide treatment. A and B: Pre-treatment images; C and D: Post-treatment images.

Figure 4

Hematoxylin-eosin staining indicated the possibility of cancer, and further immunohistochemistry was performed. The tumor was regarded as a highly differentiated neuroendocrine tumor according to the results. The tumor was positive for Syn, AE1/AE3, CD34, CK7, CK8, CD56, and CK19 (magnification, 200 ×).

Figure 5

Positron emission tomography-computed tomography was carried out after surgery and showed that the margin of the low-density shadow in the right hepatic lobe was more active and more likely attributable to postoperative changes. No other obvious abnormalities were observed.