Published online Sep 15, 2020. doi: 10.4251/wjgo.v12.i9.1031
Peer-review started: April 8, 2020
First decision: May 5, 2020
Revised: May 13, 2020
Accepted: August 15, 2020
Article in press: August 15, 2020
Published online: September 15, 2020
Processing time: 154 Days and 19.5 Hours
Neuroendocrine tumors (NETs), mostly originating from bronchopulmonary and gastrointestinal sites, are uncommon low-malignancy tumors that affect 6.98/100000 individuals annually, however, the incidence rate was in upward trend from 2000 to 2014 worldwide. Primary hepatic NETs (PHNETs), a group of NENs, are extremely rare and account for only 0.3% of all NETs. There are only few case reports about PHNETs in the literature. The lack of large sample and multicenter research results in poor diagnostic and therapeutic approaches.
Longer-term, larger sample multi-center studies are urgently needed to figure out the clinicopathological features and prognostic factors of PHNETs to facilitate their diagnosis and treatment.
This study aimed to explore the clinical characteristics, diagnosis, treatment of PHNETs and risk factors related to survival.
We analyzed the clinical data, imaging features, immunohistochemistry results, and treatment efficacy of 40 patients who were pathologically diagnosed with PHNETs from January 1, 2014 to November 15, 2019, and the survival analysis was performed to identify the risk factors for survival.
The main symptoms and signs included intermittent abdominal pain (19 patients, 47.5%) and bloating (8 patients, 20.0%). The positive rates for tumor markers included 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%). The results of immunohistochemistry staining revealed 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 . Finally, the overall survival rate was significantly related to the tumor grade, AE1/AE3, and Ki-67.
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 rate, but they can inhibit the growth of tumors, and relieve clinical symptoms.
The results of the study display the features of PHNETs patients, and reveal the relationship of the clinicopathological features and treatments with overall survival, which may facilitate the diagnosis and treatment of PHNETs.