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.

Pancreatic endocrine disorders and multiple endocrine neoplasia

2020 ◽  
pp. 2449-2463
Author(s):  
B. Khoo ◽  
T.M. Tan ◽  
S.R. Bloom
Keyword(s):  
Kinase Inhibitors ◽  
Neuroendocrine Tumours ◽  
Mammalian Target Of Rapamycin ◽  
Peptide Receptor Radionuclide Therapy ◽  
Mtor Inhibitors ◽  
Somatostatin Analogues ◽  
Endocrine Disorders ◽  
Pancreatic Neuroendocrine Tumours ◽  
Islet Cell Tumours ◽  
Cancer Syndromes

Pancreatic neuroendocrine tumours (islet-cell tumours) are rare and usually sporadic, but they may be associated with complex familial endocrine cancer syndromes. Recognized types of pancreatic neuroendocrine tumours are those that are non-functioning (often advanced at diagnosis and presenting with mass effects due to the absence of symptoms attributable to hormone hypersecretion), insulinoma (the most frequent type), and others including gastrinoma, VIPoma, and glucagonoma. The following should be considered in addition to the symptomatic treatments: surgical resection—the only curative treatment, but not possible in many cases; tyrosine kinase inhibitors which inhibit specific kinases involved in tumour cell proliferation, growth, and angiogenesis; mammalian Target of Rapamycin (mTOR) inhibitors; peptide-receptor radionuclide therapy (radiolabelled somatostatin analogues).

scholarly journals Sequence of therapy and survival in patients with advanced pancreatic neuroendocrine tumours

2020 ◽  
Vol 27 (4) ◽  
Author(s):  
E. S. Tsang ◽  
J.M. Loree ◽  
C. Speers ◽  
H.F. Kennecke
Keyword(s):  
Neuroendocrine Tumours ◽  
Progression Free Survival ◽  
Somatostatin Analogues ◽  
Treatment Modalities ◽  
Primary Role ◽  
First Line ◽  
Optimal Sequence ◽  
Systemic Treatments ◽  
Pancreatic Neuroendocrine Tumours ◽  
Line Of Therapy

Background Pancreatic neuroendocrine tumours (pnets) often present as advanced disease. The optimal sequence of therapy is unknown. Methods Sequential patients with advanced pnets referred to BC Cancer between 2000 and 2013 who received 1 or more treatment modalities were reviewed, and treatment patterns, progression-free survival (pfs), and overall survival (os) were characterized. Systemic treatments included chemotherapy, small-molecule therapy, and peptide receptor radiotherapy. Results In 66 cases of advanced pnets, median patient age was 61.2 years (25%–75% interquartile range: 50.8–66.2 years), and men constituted 47% of the group. First-line therapies were surgery (36%), chemotherapy (33%), and somatostatin analogues (32%). Compared with first-line systemic therapy, surgery in the first line was associated with increased pfs and os (20.6 months vs. 6.3 months and 100.3 months vs. 30.5 months respectively, p < 0.05). In 42 patients (64%) who received more than 1 line of therapy, no difference in os or pfs between second-line therapies was observed. Conclusions Our results confirm the primary role of surgery for advanced pnets. New systemic treatments will further increase options.

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.

Tumour Size is not a Reliable Criterion for Resection of Patients with Non-Secreting Pancreatic Neuroendocrine Tumours: Results of an International, Multi-Centre, Operative Cohort

2017 ◽  
Vol 152 (5) ◽  
pp. S1234-S1235
Author(s):  
Logan Mills ◽  
Panagiotis Drymousis ◽  
Yogesh Vashist ◽  
Christoph Burdelski ◽  
Andreas Prachalias ◽  
...  
Keyword(s):  
Neuroendocrine Tumours ◽  
Tumour Size ◽  
Reliable Criterion ◽  
Pancreatic Neuroendocrine Tumours
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