Survival in Patients with Neuroendocrine Tumours of the Small Intestine: Nomogram Validation and Predictors of Survival

Abstract

Neuroendocrine tumours of the small intestine (SI-NETs) are rare and heterogeneous. There is an unmet need for prognostication of disease course and to aid treatment strategies. A previously developed nomogram based on clinical and tumour characteristics aims to predict disease-specific survival (DSS) in patients with a SI-NET. We aimed to validate the nomogram and identify predictors of survival. Four hundred patients with a grade 1 or 2 SI-NET were included, between January 2000 and June 2016. Predicted 5- and 10-year survival was compared to actual DSS. Multivariable analysis identified predictors for actual DSS. We found that in low-, medium- and high-risk groups 5-year nomogram DSS vs. actual DSS was 0.86 vs. 0.82 (p < 0.001), 0.52 vs. 0.71 (p < 0.001) and 0.26 vs. 0.53 (p < 0.001), respectively. Ten-year nomogram DSS vs. actual DSS was 0.68 vs. 0.69 (p < 0.001), 0.40 vs. 0.50 (p < 0.001) and 0.20 vs. 0.35 (p < 0.001), respectively. Age, WHO-performance score of 2, Ki-67 index ≥10, unknown primary tumour, CgA > 6x ULN and elevated liver tests were identified as independent predictors for a worse DSS. This shows that the nomogram was able to differentiate, but underestimated DSS for patients with a SI-NET. Improvement of prognostication incorporating new emerging biomarkers is necessary to adequately estimate survival.

Biliary Neuroendocrine Neoplasms: Analysis of Prognostic Factors and Development and Validation of a Nomogram

“…The nomogram rose in response to the proper time and conditions based on these systems, including effective variables to enhance predictive ability. Regarding the nomogram in NENs, preceding studies have demonstrated its predictive value in the OS of gastric neuroendocrine neoplasms ( 26 ), the pancreas ( 27 , 28 ), the small intestine ( 29 , 30 ), the rectum ( 31 ), and the digestive system ( 32 ). These outcomes all signal that a specific, clinically applicable nomogram can accurately estimate the prognosis of patients with NENs.…”

Ileal neuroendocrine tumor: diagnosis by endoscopic capsule

“…After a careful revision of the small bowel diseases small bowel diseases excised tumor with the loco-regional lymph nodes, the patient underwent an enlargement of the surgical margin and lymphadenectomy. NETs were considered rare; however, with the advancement and availability of new diagnostic modalities such as the endoscopic capsule, an increasing number of this neoplasia has been diagnosed (Ahmed, 2020;Deguelte et al, 2020;Folkestad et al, 2020;Levy et al, 2020).…”

“…More useful diagnostic resources include determination of CgA, SYP, neuron-specific enolase (NSE), and 5-hydroxy indole acetic acid (in cases of carcinoid syndrome); CE and balloon-assisted or spiral endoscopy; and imaging study as SRS (Octreoscan), Ga-DOTATATE PET/CT or 111In-TPA-Octreotide scan (Ahmed, 2020;Deguelte et al, 2020;Levy et al, 2020;Pérez-Cuadrado-Robles et al, 2020).…”

“…Promisors in monitoring the disease are circulating tumor cells or tumor transcripts (Kim et al, 2020). The estimated detection rate of small-bowel tumors by capsule is from 2% to 9%, and capsule retention is rare (up to 2.6% of cases), more often in intestinal obstruction (Levy et al, 2020). Folkestad et al, reviewed data of 186 patients with SI-NETs and median age of 68 years, 54.3% men, and most of them underwent surgery for curative purposes or to prolong overall survival.…”

“…Neuroendocrine tumors (NETs) origin in enterochromaffin cells of the gastrointestinal (GI) tract, and small intestinal NETs (SI-NETs) arise from the submucosal crypts (Ahmed, 2020). Even with a slow growth, they usually have metastases at the time of diagnosis (Deguelte et al, 2020;Folkestad et al, 2020, Levy et al, 2020. There is a genetic influence in their etiology associated with deleting of the tumor suppressor gene PLCβ3 that causes uncontrolled growth of the neuroendocrine cells (Kamei et al, 2020).…”