Development of a nomogram model to predict survival outcomes in patients with primary hepatic neuroendocrine tumors based on SEER database

Ziteng Zhang,Guobao Li,Zhiwei Li,Zhiyan Li,Xin Zhao,Yao Liu,Youchun Wu

doi:10.1186/s12885-021-08337-y

Abstract

BackgroundPrimary hepatic neuroendocrine tumors (PH-NETs) are extremely rare and unknown. Because of its rarity, its prognosis features and influencing factors are not well established.MethodsData of 140 patients with PH-NETs diagnosed in the SEER database from 1975 to 2016 were collected. The demographics and clinic-pathological features were described. By using propensity-score matching (PSM) analysis, three associated cohorts were selected to describe the malignancy of PH-NETs and univariate analysis was conducted. Then, multivariate Cox analyses were performed and a predicting nomograph was constructed. C-index, receiver operating characteristic (ROC) curve and calibration curves were used to evaluate the predictive value of nomogram.ResultsThe overall survival outcomes of PH-NETs were superior to hepatocellular carcinoma (HCC) with a mean survival time 30.64 vs 25.11 months (p = 0.052), but inferior to gastrointestinal tract neuroendocrine tumors in situ (GI-NETs in situ) with a mean survival time 30.64 vs 41.62 months (p = 0.017). With reference to gastrointestinal neuroendocrine tumors with liver metastasis (GI-NETs-LM), GI-NETs-LM had better outcomes in short time (1-year survival rate: 64.75% vs 56.43%) but was worse in long time (5-year survival rate: 8. 63% vs 18.57%). Multivariate Cox analyses showed that tumor grade and surgery were two independent factors for prognosis of the patients (p < 0.00). Tumor grade and surgery were used to construct the predicting nomogram. The C-index was 0.79 (95%CI = 0.75–0.83). The area under curve (AUC) values in ROC were 0.868 in 1-year and 0.917 in 3-year survival and the calibration curves showed good consistency.ConclusionsThe overall prognosis PH-NETs is generally favorable, better than HCC and GI-NETs-LM in long term. Preoperative biopsy and complete pathological diagnosis were recommended. Radical surgical intervention including transplantation was the first choice in PH-NETs therapy.

Highlights

Primary hepatic neuroendocrine tumors (PH-Neuroendocrine tumors (NETs)) are extremely rare and unknown
Neuroendocrine tumors (NETs) cover a wide range of neoplasms that originate in the neuroendocrine cells that spread throughout the body
Study population In order to enroll primary hepatic neuroendocrine tumors (PH-NETs) patients, the primary site was defined by the following International Classification of Diseases for Oncology (ICD-O-3) codes: liver (C22.0)

Summary

Introduction

Primary hepatic neuroendocrine tumors (PH-NETs) are extremely rare and unknown. Recent study have suggested a 6.4-fold increase in its incidence rate from 1973 (1.09 per 100,000) to 2012 (6.98 per 100,000) [1, 2]. According to a recent United States population-based studies, gastrointestinal tract (GI) was the most frequently occurring site in NETs, followed by lung. Primary hepatic neuroendocrine tumors (PH-NETs) are rare neuroendocrine tumors, representing 0.4% of all NETs, and were first described by Edmonson in 1958 [3, 4]. Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) were the first two most common primary malignant hepatic tumors (PMHTs). The most common PMHTs were neuroendocrine tumor and lymphoma which were infrequent and poorly known [5]

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