Healthcare cost by primary tumour, functioning status and treatment among patients with metastatic neuroendocrine tumours: the LyREMeNET study

Author(s):  
Marine Perrier ◽  
Stéphanie Polazzi ◽  
Annie Lemelin ◽  
Violaine Fernandez ◽  
Stéphanie Labonne ◽  
...  
2018 ◽  
Vol 107 (3) ◽  
pp. 292-304 ◽  
Author(s):  
Faidon-Marios Laskaratos ◽  
Martin Walker ◽  
Dominic Wilkins ◽  
Alexander Tuck ◽  
Shashank Ramakrishnan ◽  
...  

Background: Small intestinal neuroendocrine tumours (SI NETs) represent 30–50% of small bowel neoplasms and often present at an advanced stage. To date, there is relatively limited literature regarding prognostic factors affecting overall survival (OS) in stage IV disease. In addition, the prevalence of mesenteric fibrosis (MF) in SI NETs and its effect on OS have not been sufficiently explored in the literature. Aim: The primary aim of this study was to perform a large-scale survival analysis in an institutional cohort of 387 patients with metastatic (stage IV) SI NETs. The secondary aim was to provide epidemiological information regarding the prevalence of MF and to evaluate its effect on OS. Results: The median OS was 101 months (95% CI 84, 118). Age > 65 years, mesenteric metastases with and without desmoplasia, liver metastases, carcinoid heart disease (CHD) and bone metastases were associated with a significantly shorter OS, while primary tumour resection was predictive of a longer OS. The benefit of surgical resection was limited to symptomatic patients. MF was present in approximately 50% of patients with mesenteric lymphadenopathy. Elevated urinary 5-HIAA levels correlated strongly with the presence of CHD (p < 0.001) and to a lesser extent (p = 0.02) with MF. MF and CHD did not usually co-exist, suggesting that different mechanisms are likely to be involved in the development of these fibrotic complications. Conclusions: This study has identified specific prognostic factors in a large cohort of 387 patients with advanced SI NETs and has provided useful epidemiological data regarding carcinoid-related fibrotic complications.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e14703-e14703
Author(s):  
Laeeq Malik ◽  
Yu Jo Chua ◽  
Nadeem Butt ◽  
Desmond Yip

e14703 Background: Neuroendocrine tumours (NETs) have been regarded as indolent tumors with significantly variable clinical behavior. Limited information is available on long-term clinical outcome and clinically applicable prognostic factors.We performed a retrospective review of NETS managed in the Australian Capital Territory (ACT) over a 12-year period,with examination of epidemiology and various prognostic clinicopathologic factors. Methods: This multicenter analysis included patients in ACT and surrounding New South Wales treated with histologically proven neuroendocrine tumor (lung carcinoids excluded). The cases were identified from hospital databases. Data was analysed according to epidemiological, clinical and histopathological characteristics. Results: The cohort of 107 patients showed slight male predominance. Median age at diagnosis was 62 years and tumour size of 1.2 cm. The most common primary tumour site was jejunum/ileum (32%) followed by rectum (22%) and pancreas (11.2 %). Most patients had localised disease at initial diagnosis (n- 73/107 (68%). Distant metastases were seen in 32% (n-34/107) on initial staging with liver being most common site. Most patients were symptomatic at diagnosis while 22.4% cases were found incidentally. Second malignancies in particular of gastrointestinal origin were diagnosed in 33.6% (n-36/107). Surgical debulking was the most common treatment (59.8%) while 18% had multi-modality therapy. At a median followup of 25 months from diagnosis, 76 patients (78%) were still alive. Median time to first relapse was 15 months. 5 year survival rate was 75% for NETs originating from jejunum/ilieum on Kaplan-Meier analysis. Increasing age, tumor size, male gender, high histological grade, high Ki 67 index, raised plasma chromogranin A and urine 5 HIAA at the time of diagnosis were associated with shorter 5-year survival. Conclusions: The epidemiologic characteristics and long-term outcome in our series was comparable to reported studies from other centers. This analysis confirmed some important prognostic factors that could be considered for risk stratification and therapeutic management in patients with NETs.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 4599-4599
Author(s):  
D. Hörsch ◽  
V. Prasad ◽  
V. Ambrosini ◽  
M. Hommann ◽  
S. Fanti ◽  
...  

4599 Background: This bi-centric study aimed at determining the role of receptor PET/CT using 68Ga-DOTA-NOC in the detection of undiagnosed primary sites of neuroendocrine tumours (NETs). Methods: Overall 59 patients (M: F 33:26, age 65±9 yr) with documented NET and unknown primary, were enrolled. PET/CT was performed after injection of approximately 100 MBq (46–260 MBq) of 68Ga-DOTA-NOC. The maximum standardised uptake values (SUVmax) were calculated and compared with SUVmax in known pancreatic NET (pNET) and ileum / jejunum / duodenum (SI-NET). The results of PET/CT were also correlated with CT alone. Results: In 35/59 (59%) of patients, 68Ga-DOTA-NOC PET/CT localised the site of the primary: ileum/jejunum (14), pancreas (16), rectum (2), lungs (2) and paraganglioma (1). CT alone (on retrospective analyses) confirmed the findings in 12/59 (20%) patients. The mean SUVmax of identified previously unknown pNET and SI-Net were 18.6 ± 9.8 (range 7.8–34.8) and 9.1± 6.0 (range 4.2–27.8), respectively. SUVmax in patients with previously known pNET and SI-NET were 26.1± 14.5 (range 8.7–42.4) and 11.3±3.7 (range 5.6- 17.9). The SUVmax of the unknown pNET and SI-NET were significantly lower (p< 0.05) as compared to the ones with known primary tumour sites. 19% of the patients had high grade, and 81% low grade NET. In 4/59 patients the primary tumour was subsequently resected (2 pancretic, one ileal and one rectal tumour). Conclusions: Our data indicate that 68Ga- DOTA-NOC PET/CT is highly superior to 111In Octreoscan (17% detection rate for CUP according to literature) and can play a major role in the management of patients with CUP-NET. No significant financial relationships to disclose.


2018 ◽  
Vol 25 (3) ◽  
pp. 245-254 ◽  
Author(s):  
Anela Blažević ◽  
Wouter T Zandee ◽  
Gaston J H Franssen ◽  
Johannes Hofland ◽  
Marie-Louise F van Velthuysen ◽  
...  

Mesenteric fibrosis (MF) surrounding a mesenteric mass is a hallmark feature of small intestinal neuroendocrine tumours (SI-NETs). Since this can induce intestinal obstruction, oedema and ischaemia, prophylactic resection of the primary tumour and mesenteric mass is often recommended. This study assessed the predictors for mesenteric metastasis and fibrosis and the effect of MF and palliative surgery on survival. A retrospective analysis of 559 patients with pathologically proven SI-NET and available CT-imaging data was performed. Clinical characteristics, presence of mesenteric mass and fibrosis on CT imaging and the effect of palliative abdominal surgery on overall survival were assessed. We found that MF was present in 41.4%. Older age, 5-HIAA excretion ≥67 μmol/24 h, serum CgA ≥121.5 μg/L and a mesenteric mass ≥27.5 mm were independent predictors of MF. In patients ≤52 years, mesenteric mass was less often found in women than in men (39% vs 64%,P = 0.002). Corrected for age, tumour grade, CgA and liver metastasis, MF was not a prognostic factor for overall survival. In patients undergoing palliative surgery, metastasectomy of mesenteric mass or prophylactic surgery did not result in survival benefit. In conclusion, we confirmed known predictors of MF and mesenteric mass and suggest a role for sex hormones as women ≤52 years have less often a mesenteric mass. Furthermore, the presence of MF has no effect on survival in a multivariate analysis, and we found no benefit of metastasectomy of mesenteric mass or prophylactic surgery on overall survival.


2020 ◽  
Vol 9 (8) ◽  
pp. 2502
Author(s):  
Sonja Levy ◽  
Linde M. van Veenendaal ◽  
Catharina M. Korse ◽  
Emilie C.H. Breekveldt ◽  
Wieke H.M. Verbeek ◽  
...  

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.


2013 ◽  
Vol 154 (39) ◽  
pp. 1549-1555 ◽  
Author(s):  
Andrea Uhlyarik ◽  
Zsuzsanna Pápai

Neuroendocrine neoplasms belong to the group of rare tumours. Their clinical importance may be highlighted by their high prevalence despite low incidence. Since survival rate is similar to other progressive neoplastic diseases in metastatic cases, early recognition and appropriate therapy of these neoplasms are equally important. Classification of neuroendocrine tumours is based on their pathologic characteristics according to the 2010 WHO recommendation. Non-functioning tumours cause local symptoms due to their mass effect, while functioning tumours produce well-defined endocrine syndromes. Among laboratory tests, serum chromogranin-A is considered the most important biomarker of both non-functioning and functioning neuroendocrine tumours. Localization of these tumours includes the use of conventional diagnostic imaging, endoscopic examinations, and functional imaging studies. With respect to treatment, elimination of the primary tumour remains one of the most important issues. In advanced cases of the disease metastasectomy, interventional radiologic methods, medical treatment and endoradiotherapy can be used. The aim of this review is to summarize briefly the symptoms, diagnostic methods and treatment options of neuroendocrine tumours. Orv. Hetil., 2013, 154, 1549–1555.


2016 ◽  
Vol 23 (9) ◽  
pp. 711-726 ◽  
Author(s):  
Helen C Miller ◽  
Adam E Frampton ◽  
Anna Malczewska ◽  
Silvia Ottaviani ◽  
Euan A Stronach ◽  
...  

Novel molecular analytes are needed in small bowel neuroendocrine tumours (SBNETs) to better determine disease aggressiveness and predict treatment response. In this study, we aimed to profile the global miRNome of SBNETs, and identify microRNAs (miRNAs) involved in tumour progression for use as potential biomarkers. Two independent miRNA profiling experiments were performed (n=90), including primary SBNETs (n=28), adjacent normal small bowel (NSB; n=14), matched lymph node (LN) metastases (n=24), normal LNs (n=7), normal liver (n=2) and liver metastases (n=15). We then evaluated potentially targeted genes by performing integrated computational analyses. We discovered 39 miRNAs significantly deregulated in SBNETs compared with adjacent NSB. The most upregulated (miR-204-5p, miR-7-5p and miR-375) were confirmed by qRT-PCR. Two miRNAs (miR-1 and miR-143-3p) were significantly downregulated in LN and liver metastases compared with primary tumours. Furthermore, we identified upregulated gene targets for miR-1 and miR-143-3p in an existing SBNET dataset, which could contribute to disease progression, and show that these miRNAs directly regulate FOSB and NUAK2 oncogenes. Our study represents the largest global miRNA profiling of SBNETs using matched primary tumour and metastatic samples. We revealed novel miRNAs deregulated during SBNET disease progression, and important miRNA–mRNA interactions. These miRNAs have the potential to act as biomarkers for patient stratification and may also be able to guide treatment decisions. Further experiments to define molecular mechanisms and validate these miRNAs in larger tissue cohorts and in biofluids are now warranted.


ISRN Oncology ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Rajaventhan Srirajaskanthan ◽  
A. Ahmed ◽  
A. Prachialias ◽  
P. Srinivasan ◽  
N. Heaton ◽  
...  

Introduction. Small bowel neuroendocrine tumours (NETs) are the most common type of gastrointestinal neuroendocrine tumours. The incidence and prevalence of these tumours are on the rise. The aims of this study were to determine prognostic clinicopathological features and whether the ENETS TNM staging system predicts prognosis and also. Method. Clinical data was collected retrospectively from 138 patients with histologically proven small bowel NETs managed at King’s College Hospital. Histology was reviewed and small bowels tumours, were staged according to the ENETS TNM staging system. Results. Median age was 65 years (range 29–87). The 5-year survival was 79.5% and the 10-year survival was 48.5%. Resection of the primary tumour was associated with improved survival (120 versus 56 months, P<0.05). On multivariate analysis prognostic factors were primary tumour resection and not having a carcinoid heart disease. TNM staging significantly separated survival of stage 2 and stage 3 from stage 4 NETs. Conclusion. Small bowel primary tumour resection and not having carcinoid heart disease are prognostic factors. The ENETS TNM staging and grading system appears to be of prognostic relevance to small bowel NETs.


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