scholarly journals Chemotherapy for Well-Differentiated Pancreatic Neuroendocrine Tumours with a Ki-67 Index ≥10%: Is There a More Effective Antitumour Regimen? A Retrospective Multicentre Study of the French Group of Endocrine Tumours (GTE)

2017 ◽  
Vol 106 (1) ◽  
pp. 38-46 ◽  
Author(s):  
Guillaume Roquin ◽  
Eric Baudin ◽  
Catherine Lombard-Bohas ◽  
Guillaume Cadiot ◽  
Sophie Dominguez ◽  
...  
Keyword(s):  
Multicentre Study ◽  
Neuroendocrine Tumours ◽  
Ki 67 ◽  
Endocrine Tumours ◽  
French Group ◽  
Pancreatic Neuroendocrine Tumours ◽  
Well Differentiated

scholarly journals Identifying Prognostic Factors for Well-Differentiated Metastatic Pancreatic Neuroendocrine Tumours: A Retrospective International Multicentre Cohort Study

2018 ◽  
Vol 107 (4) ◽  
pp. 315-323 ◽  
Author(s):  
Paula Jiménez-Fonseca ◽  
Sebastian Krug ◽  
Gianluca Tamagno ◽  
Felipe Fierro Maya ◽  
Antonio Monléon Getino ◽  
...  
Keyword(s):  
Alkaline Phosphatase ◽  
Multivariate Analysis ◽  
Cohort Study ◽  
Neuroendocrine Tumours ◽  
Curative Surgery ◽  
Risk Of Death ◽  
Pancreatic Neuroendocrine Tumours ◽  
Synchronous Metastases ◽  
Well Differentiated ◽  
The Impact

Pancreatic neuroendocrine tumours (pNETs) represent rare neoplasms of all NETs often presenting without functional activity. Many sporadic non-functioning pNET patients are already metastatic at the time of diagnosis, and the therapeutic approach to such patients is mostly palliative. In this international, multicentre, retrospective cohort study, we assessed the prognostic value of a set of anthropometric, clinical, biochemical, radiological and pathological parameters at baseline and the impact of the therapeutic strategies on the survival of patients with sporadic grade 1/2, stage IV, non-functioning pNETs. Three hundred and twelve consecutive patients diagnosed between 1993 and 2010 were included. The median overall survival (OS) was 6.6 years and survival at 5 and 10 years was 62 and 34% respectively. On univariate analysis, Eastern Cooperative Oncology Group (ECOG) status ≥2, grade 2, bilobar hepatic metastases, synchronous metastases, and high chromogranin A, alkaline-phosphatase and lactic-dehydrogenase were associated with a significant reduction of OS. Palliative/curative surgery and loco-regional hepatic interventions were significant factors improving OS. On multivariate analysis, ECOG status ≥2, synchronous metastases, Ki-67 ≥10%, and high alkaline-phosphatase correlated significantly with an increased risk of death. Both palliative/curative surgery and loco-regional hepatic interventions had a positive impact on OS. Although most parameters did not prove to be independent OS predictors at multivariate analysis, they showed a tendency towards that. Future prospective studies including larger patient populations may give greater clarity. We believe the integration of these parameters has the potential to provide a reliable prognostic score for the stratification of patients with sporadic well-differentiated metastatic non-functioning pNETs.

Efficacy and Safety of Sunitinib in Patients with Well-Differentiated Pancreatic Neuroendocrine Tumours

2018 ◽  
Vol 107 (3) ◽  
pp. 237-245 ◽  
Author(s):  
Eric Raymond ◽  
Matthew H. Kulke ◽  
Shukui Qin ◽  
Xianjun Yu ◽  
Michael Schenker ◽  
...  
Keyword(s):  
Neuroendocrine Tumours ◽  
Phase Iii ◽  
Choi Criteria ◽  
Treatment Naïve ◽  
Phase Iii Study ◽  
Previously Treated Patients ◽  
Pancreatic Neuroendocrine Tumours ◽  
Previously Treated ◽  
Well Differentiated ◽  
Phase Iv

Background: In a phase III study, sunitinib led to a significant increase in progression-free survival (PFS) versus placebo in patients with pancreatic neuroendocrine tumours (panNETs). This study was a post-marketing commitment to support the phase III data. Methods: In this ongoing, open-label, phase IV trial (NCT01525550), patients with progressive, advanced unresectable/metastatic, well-differentiated panNETs received continuous sunitinib 37.5 mg once daily. Eligibility criteria were similar to those of the phase III study. The primary endpoint was investigator-assessed PFS per Response Evaluation Criteria in Solid Tumours v1.0 (RECIST). Other endpoints included PFS per Choi criteria, overall survival (OS), objective response rate (ORR), and adverse events (AEs). Results: Sixty-one treatment-naive and 45 previously treated patients received sunitinib. By March 19, 2016, 82 (77%) patients had discontinued treatment, mainly due to disease progression. Median treatment duration was 11.7 months. Investigator-assessed median PFS per RECIST (95% confidence interval [CI]) was 13.2 months (10.9–16.7): 13.2 (7.4–16.8) and 13.0 (9.2–20.4) in treatment-naive and previously treated patients, respectively. ORR (95% CI) per RECIST was 24.5% (16.7–33.8) in the total population: 21.3% (11.9–33.7) in treatment-naive and 28.9% (16.4–44.3) in previously treated patients. Median OS, although not yet mature, was 37.8 months (95% CI, 33.0–not estimable). The most common treatment-related AEs were neutropenia (53.8%), diarrhoea (46.2%), and leukopenia (43.4%). Conclusions: This phase IV trial confirms sunitinib as an efficacious and safe treatment option in patients with advanced/metastatic, well-differentiated, unresectable panNETs, and supports the phase III study outcomes. AEs were consistent with the known safety profile of sunitinib.

Pancreatic Neuroendocrine Tumours: The Role of Endoscopic Ultrasound Biopsy in Diagnosis and Grading Based on the WHO 2017 Classification

2019 ◽  
Vol 37 (4) ◽  
pp. 325-333 ◽  
Author(s):  
Milena Di Leo ◽  
Laura Poliani ◽  
Daoud Rahal ◽  
Francesco Auriemma ◽  
Andrea Anderloni ◽  
...  
Keyword(s):  
Endoscopic Ultrasound ◽  
Neuroendocrine Tumours ◽  
Histological Grade ◽  
Accurate Method ◽  
Biopsy Material ◽  
Ki 67 ◽  
Surgical Specimen ◽  
Study Population ◽  
Pancreatic Neuroendocrine Tumours

Background: One of the controversial issues in the diagnosis of pancreatic neuroendocrine tumours (pNETs) is the accurate prediction of their clinical behaviour. Objectives: The aim of the study was to evaluate the role of endoscopic ultrasound (EUS) biopsy in the diagnosis and grading of pNETs in a certified ENETS Center. Methods: A prospectively maintained database of EUS biopsy procedures was retrospectively reviewed to identify all consecutive patients referred to a certified ENETS Center with a suspicion of pNET between June 2014 and April 2017. The cytological and/or histological specimens were stained and the Ki-67 labeling index was evaluated. In patients undergoing surgery, the grade obtained with EUS-guided biopsy was compared with the final histological grade. The grade was evaluated according to the 2017 WHO classifications and grading. Results: The study population included 59 patients. EUS biopsy material reached an adequacy of 98.3% and was adequate for Ki-67 evaluation in 84.7% of cases. Twenty-nine patients (49.2%) underwent surgery. Of these, 25 patients had Ki-67 evaluated on EUS biopsy: the agreement between EUS biopsy grading and surgical specimen grading was 84%. Conclusion: EUS biopsy is an accurate method for the diagnosis and grading of pNETs based on the WHO 2017 Ki-67 labelling scheme.

scholarly journals Serotonin-Secreting Neuroendocrine Tumours of the Pancreas

2020 ◽  
Vol 9 (5) ◽  
pp. 1363
Author(s):  
Anna Caterina Milanetto ◽  
Matteo Fassan ◽  
Alina David ◽  
Claudio Pasquali
Keyword(s):  
Liver Metastases ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumours ◽  
Tumour Size ◽  
Somatostatin Analogues ◽  
High Serum ◽  
Resective Surgery ◽  
Alive With Disease ◽  
Pancreatic Neuroendocrine Tumours ◽  
Well Differentiated

Background: Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels). Methods: Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986–2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels. Results: Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38–69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5–10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4–70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. One patient is alive with disease 117 months after observation. All the others died from disease progression after a follow-up within 158 months. Conclusions: Primary 5-HT-secreting pNETs are mostly metastatic to the liver; patients are not amenable to resective surgery. Despite high 5-HIAA urinary levels, few patients present with carcinoid syndrome. A five-year survival rate of 42.9% may be achieved with multimodal treatment.

scholarly journals Glyoxalase 1 copy number variation in patients with well differentiated gastro-entero-pancreatic neuroendocrine tumours (GEP-NET)

Oncotarget ◽  
2017 ◽  
Vol 8 (44) ◽  
pp. 76961-76973 ◽  
Author(s):  
Mingzhan Xue ◽  
Alaa Shafie ◽  
Talha Qaiser ◽  
Nasir M. Rajpoot ◽  
Gregory Kaltsas ◽  
...  
Keyword(s):  
Copy Number Variation ◽  
Copy Number ◽  
Neuroendocrine Tumours ◽  
Glyoxalase 1 ◽  
Number Variation ◽  
Pancreatic Neuroendocrine Tumours ◽  
Well Differentiated
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