Gastric neuroendocrine neoplasias: manifestations and comparative outcomes

Abstract

Although gastric neuroendocrine neoplasias (gNEN) are an orphan disease, their incidence is rising. The heterogeneous clinical course powers the ongoing discussion of the most appropriate classification system and management. Prognostic relevance of proposed classifications was retrospectively analysed in 142 patients from a single tertiary referral centre. Baseline, management and survival data were acquired for statistical analyses. The distribution according to the clinicopathological typification was gNEN-1 (n = 86/60.6%), gNEN-2 (n = 7/4.9%), gNEN-3 (n = 24/16.9%) and gNEN-4 (n = 25/17.6%), while hypergastrinemia-associated gNEN-1 and -2 were all low-grade tumours (NET-G1/2), formerly termed sporadic gNEN-3 could be subdivided into gNEN-3 with grade 1 or 2 and gNEN-4 with grade 3 (NEC-G3). During follow-up 36 patients died (25%). The mean overall survival (OS) of all gNEN was 14.2 years. The OS differed statistically significant across all subgroups with either classification system. According to UICC 2017 TNM classification, OS differed for early and advanced stages, while WHO grading indicated poorer prognosis for NEC-G3. Cox regression analysis confirmed the independent prognostic validity of either classification system for survival. Particularly careful analysis of the clinical course of gNEN-1 (ECLomas, gastric carcinoids) confirmed their mostly benign, but recurrent and extremely slowly progressive behaviour with low risk of metastasis (7%) and an efficient long-term control by repetitive endoscopic procedures. Our study provides evidence for the validity of current classifications focusing on typing, grading and staging. These are crucial tools for risk stratification, especially to differentiate gNEN-1 as well as sporadic gNET and gNEC (gNEN-3 vs -4).

Endoscopic management of subepithelial lesions including neuroendocrine neoplasms: European Society of Gastrointestinal Endoscopy (ESGE) Guideline

“…In the stomach NENs can present as multifocal subepithelial lesions. These are usually diagnosed by standard mucosal biopsy techniques [12]. There is a stark contrast between the usually aggressive nature of esophageal NENs (often high grade small-cell type in nature) and the mostly more indolent nature of gastric NENs, so that NENs from each GI site warrant separate discussion (▶ Table 2).…”

Clinicopathological Features and Postoperative Survival Analysis of Gastric Carcinoma with Neuroendocrine Differentiation

“…Some scholars have found that the postoperative survival time of gastric cancer patients with neuroendocrine differentiation (NED) is shorter than that of patients without NED, and the difference is statistically significant. This might imply that neuroendocrine differentiation plays a role in promoting gastric cancer progression [ 12 , 13 ]. Some scholars believe that the survival time of tumor patients with high expression of neuroendocrine markers CgA and Syn is significantly lower than that of patients with low expression.…”

ENETS standardized (synoptic) reporting for endoscopy in neuroendocrine tumors

“…Gastric atrophy associated with chronic H . pylori ‐infection may also be a factor in some cases 19–21 . Both type 1 and type 2 gNEN typically arise as multiple gastric polyps (See Case Study 1 and Figure 1) or subepithelial lesions (SEL), type 2 being quite rare while type 1 is by far the most frequent gNEN 16–18 …”

“…[16][17][18] Gastric atrophy associated with chronic H. pylori-infection may also be a factor in some cases. [19][20][21] Both type 1 and type 2 gNEN typically arise as multiple gastric polyps (See Case Study 1 and Figure 1) or Tumor is also known in the literature as small cell carcinoma of the oesophagus.…”