TY - JOUR
T1 - Survival in patients with neuroendocrine tumours of the small intestine
T2 - Nomogram validation and predictors of survival
AU - Levy, Sonja
AU - van Veenendaal, Linde M.
AU - Korse, Catharina M.
AU - Breekveldt, Emilie C.H.
AU - Verbeek, Wieke H.M.
AU - Vriens, Menno R.
AU - Kuhlmann, Koert F.D.
AU - van den Berg, José G.
AU - Valk, Gerlof D.
AU - Tesselaar, Margot E.T.
N1 - Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2020/8
Y1 - 2020/8
N2 - 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.
AB - 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.
KW - Neuroendocrine tumours
KW - Nomogram
KW - Prediction model
KW - Prognosis
KW - Small‐intestine
UR - http://www.scopus.com/inward/record.url?scp=85111945584&partnerID=8YFLogxK
U2 - 10.3390/jcm9082502
DO - 10.3390/jcm9082502
M3 - Article
C2 - 32756529
AN - SCOPUS:85111945584
SN - 2077-0383
VL - 9
SP - 1
EP - 14
JO - Journal of Clinical medicine
JF - Journal of Clinical medicine
IS - 8
M1 - 2502
ER -