Clinicopathological features and prognosis of gastroenteropancreatic neuroendocrine neoplasms in a Chinese population: a large, retrospective single-centre study

Abstract

Background: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are the most common type of neuroendocrine tumors, accounting for more than half of neuroendocrine neoplasms (NENs). We performed a retrospective study in our center to investigate the clinicopathological features, risk factors of metastasis, and prognosis of GEP-NENs in a Chinese population.

Methods: Four hundred forty patients with GEP-NENs treated at the First Affiliated Hospital of Zhengzhou University between January 2011 and March 2016 were analyzed retrospectively. Multivariate logistic regression was performed to identify independent risk factors for metastasis of the tumors. The Kaplan-Meier method was used for survival analysis, and log-rank tests for comparisons among groups.

Results: Primary sites were the stomach (24.3%), rectum (24.1%), pancreas (20.5%), esophagus (12.3%), unknown primary origin (UPO-NEN) (8.0%), duodenum (6.1%). Three hundred eighty-nine of the 440 GEP-NENs cases (88.4%) were non-functional tumors, and patients had non-specific symptoms, which could have led to delay in diagnosis and treatment. Neuroendocrine tumor, neuroendocrine carcinoma, and mixed adenoendocrine carcinoma were 56.8%, 33.2% and 3.2%, respectively, of the cases. One hundred thirty (29.5%) of the tumors were G1, 120 (27.3%) G2, and 190 (43.2%) G3. The immunohistochemical positive rate of synaptophysin was 97.7% and of chromogranin 48.7%. Logistic regression analysis revealed that the diameter and pathological classification of tumors were the most important predictors for metastasis. The median survival time was 34 months for patients with well-differentiated neuroendocrine tumors grade G3 and 11 months for poorly differentiated neuroendocrine carcinoma. The median survival time of patients with localized disease, regional disease, and distant disease was 36 months, 15 month, and 6 months, respectively.

Conclusions: This study constitutes a comprehensive analysis of the clinicopathological features of GEP-NENs in a Chinese population. GEP-NENs may occur at any part of the digestive system. The diameter and pathological classification of tumor are the most important predictors for metastasis. The prognosis is poor for patients with poorly differentiated neuroendocrine cancers and distant metastases.

Keywords: Carcinoid tumor; Gastrinoma; Gastro enteropancreatic neuroendocrine tumors; Islet cell tumor; Neuroendocrine cancers; Neuroendocrine neoplasms; Neuroendocrine tumors.

Fig. 1

Kaplan-Meier analysis of overall survival. a Overall survival by histological grading. b Overall survival by age of of patients with G3 tumors. c Overall survival by sex of patient with G3 tumors. d Overall survival by site of G3 tumors. e Overall survival by differentiation of G3 tumors. f Overall survival by characteristics of metastasis of G3 tumors