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Pancreatic Neoplasms: HELP
Articles by Bruno Niederle
Based on 8 articles published since 2008

Between 2008 and 2019, B. Niederle wrote the following 8 articles about Pancreatic Neoplasms.
+ Citations + Abstracts
1 Guideline ENETS Consensus Guidelines for High-Grade Gastroenteropancreatic Neuroendocrine Tumors and Neuroendocrine Carcinomas. 2016

Garcia-Carbonero, R / Sorbye, H / Baudin, E / Raymond, E / Wiedenmann, B / Niederle, B / Sedlackova, E / Toumpanakis, C / Anlauf, M / Cwikla, J B / Caplin, M / O'Toole, D / Perren, A / Anonymous6950853. ·Medical Oncology Department, Hospital Universitario Doce de Octubre, Madrid, Spain. ·Neuroendocrinology · Pubmed #26731334.

ABSTRACT: -- No abstract --

2 Review [Benign neuroendocrine and other rare benign tumors of the pancreas]. 2008

Happel, B / Niederle, B / Puespoek, A / Ba-Ssalamah, A / Schima, W. ·Universitätsklinik für Radiodiagnostik, Medizinische Universität Wien, Währinger Gürtel 18-20, A-1090, Wien, Osterreich. brigitte.happel@meduniwien.ac.at ·Radiologe · Pubmed #18633589.

ABSTRACT: Neuroendocrine tumors (NET) of the pancreas are rare neoplasms, which arise from cells of the islets of Langerhans. The most common NET are the insulinoma, gastrinoma and hormone inactive NET. Very rare entities are the schwannoma, leiomyoma, teratoma, intrapancreatic lipoma, hemangioma and the intrapancreatic accessory spleen. Essential for therapy, which in most cases is difficult, are an exact localization and various modalities of imaging diagnostics.

3 Article Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1. 2019

Selberherr, Andreas / Koperek, Oskar / Riss, Philipp / Scheuba, Christian / Niederle, Martin B / Kaderli, Reto / Perren, Aurel / Niederle, Bruno. ·Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, Medical University, Währinger Gürtel 18-20, A-1090, Vienna, Austria. andreas.selberherr@meduniwien.ac.at. · Department of Pathology, Medical University, Währinger Gürtel 18-20, A-1090, Vienna, Austria. · Section "Endocrine Surgery", Division of General Surgery, Department of Surgery, Medical University, Währinger Gürtel 18-20, A-1090, Vienna, Austria. · Department of Anesthesiology, Medical University, Währinger Gürtel 18-20, A-1090, Vienna, Austria. · Institute of Pathology, University of Bern, Murtenstrasse 31, CH-3012, Bern, Switzerland. ·Orphanet J Rare Dis · Pubmed #30795813.

ABSTRACT: BACKGROUND: Patients with multiple endocrine neoplasia type 1 (MEN-1) develop multiple pancreatic neuroendocrine neoplasias (PNENs). Size at diagnosis and growth during follow-up are crucial parameters. According to the WHO 2017, grading is another important parameter. The impact of grading compared to size (WHO 2000) on the clinical course needs to be evaluated. METHODS: Sixty PNENs of six patients with MEN-1 were retrospectively evaluated. RESULTS: Fifty-one tumors with a diameter of < 20 mm were graded as G1. Two of 9 tumors with diameters of ≥20 mm were graded as G2. Tumor size of ≥20 mm correlated significantly with higher proliferation (p = 0.000617). Lymph node metastases were documented in two patients with a total of 19 tumors. In one patient, all 13 tumors (diameter: 0.4 to 100 mm) were classified as G1. However, metastases were documented in 9/29 lymph nodes. In the other patient, 5 tumors (3.5 to 20 mm) were classified as G1. The sixth tumor (30 mm) was classified as G2 (Ki-67: 8%). Metastases were revealed in 2/20 lymph nodes. CONCLUSIONS: Tumor size of ≥20 mm seems to correlate with more aggressive MEN-1 related pancreatic disease, regardless of individual proliferation. Tumors ≥20 mm and tumors graded as G2 should be treated surgically regardless of their size.

4 Article None 2018

Iacovazzo, Donato / Flanagan, Sarah E / Walker, Emily / Quezado, Rosana / de Sousa Barros, Fernando Antonio / Caswell, Richard / Johnson, Matthew B / Wakeling, Matthew / Brändle, Michael / Guo, Min / Dang, Mary N / Gabrovska, Plamena / Niederle, Bruno / Christ, Emanuel / Jenni, Stefan / Sipos, Bence / Nieser, Maike / Frilling, Andrea / Dhatariya, Ketan / Chanson, Philippe / de Herder, Wouter W / Konukiewitz, Björn / Klöppel, Günter / Stein, Roland / Korbonits, Márta / Ellard, Sian. ·Centre for Endocrinology, Barts and The London School of Medicine, Queen Mary University of London, EC1M 6BQ London, United Kingdom. · Institute of Biomedical and Clinical Science, University of Exeter Medical School, EX2 5DW Exeter, United Kingdom. · Department of Molecular Physiology and Biophysics, Vanderbilt University, Nashville, TN 37232. · Serviço de Endocrinologia e Diabetes, Hospital Universitário Walter Cantídio, Universidade Federal do Ceará, 60430-372 Fortaleza, Brazil. · Division of Endocrinology and Diabetes, Department of Internal Medicine, Kantonsspital St. Gallen, CH-9007 St. Gallen, Switzerland. · Section of Endocrine Surgery, Division of General Surgery, Department of Surgery, University of Vienna, A-1090 Vienna, Austria. · Division of Diabetes, Endocrinology and Metabolism, University Hospital of Basel, CH-4031 Basel, Switzerland. · Division of Endocrinology, Diabetes and Clinical Nutrition, University Hospital of Bern, Inselspital, CH-3010 Bern, Switzerland. · Department of Pathology, University of Tübingen, 72076 Tübingen, Germany. · Department of Surgery and Cancer, Imperial College London, W12 0HS London, United Kingdom. · Elsie Bertram Diabetes Centre, Norfolk and Norwich University Hospitals NHS Foundation Trust, NR4 7UY Norwich, United Kingdom. · Service d'Endocrinologie et des Maladies de la Reproduction, Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, F-94275 Le Kremlin-Bicêtre, France. · INSERM 1185, Faculté de Médicine Paris Sud, Université Paris-Saclay, F-94276 Le Kremlin-Bicêtre, France. · Department of Internal Medicine, Sector of Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumors, Erasmus MC, 3015 Rotterdam, The Netherlands. · Department of Pathology, Consultation Center for Pancreatic and Endocrine Tumors, Technical University of Munich, 81675 Munich, Germany. · Centre for Endocrinology, Barts and The London School of Medicine, Queen Mary University of London, EC1M 6BQ London, United Kingdom; m.korbonits@qmul.ac.uk. ·Proc Natl Acad Sci U S A · Pubmed #29339498.

ABSTRACT: The β-cell-enriched MAFA transcription factor plays a central role in regulating glucose-stimulated insulin secretion while also demonstrating oncogenic transformation potential in vitro. No disease-causing

5 Article Clinical presentation in insulinoma predicts histopathological tumour characteristics. 2015

Wolf, Peter / Winhofer, Yvonne / Smajis, Sabina / Anderwald, Christian-Heinz / Scheuba, Christian / Niederle, Bruno / Gessl, Alois / Luger, Anton / Krebs, Michael / Koperek, Oskar. ·Division of Endocrinology and Metabolism, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria. · Metabolic Unit, Institute of Biomedical Engineering, National Research Council, Padova, Italy. · Medical Direction, Specialized Hospital Complex Agathenhof, Micheldorf, Austria. · Department of Surgery, Medical University of Vienna, Vienna, Austria. · Department of Pathology, Medical University of Vienna, Vienna, Austria. ·Clin Endocrinol (Oxf) · Pubmed #25817061.

ABSTRACT: BACKGROUND: Insulinomas are rare neuroendocrine tumours (NETs) of the pancreas, characterized clinically by neuroglycopenic symptoms during periods of substrate deficiency. The gold standard test for diagnosing an insulinoma is a 72-h fast. However, the prognostic value of parameters in the standardized 72-h fast on histopathological tumour criteria and clinical presentation has not been examined. METHODS: In thirty-three patients diagnosed with an insulinoma records, and data were investigated retrospectively. Histopathological tumour characteristics, including staging, grading and size, were reviewed. Grading was performed using Ki-67 index. Cut-off values for classical grading (G(clas)) were set at G1(clas) ≤ 2%, G2(clas) 3-20% & G3(clas) >20% and for modified grading (G(mod)) at G1(mod) <5%, G2(mod) 5-20% & G3(mod) >20%. RESULTS: When G(mod) criteria were applied, the initial blood glucose was lower in GII/III(mod) patients compared to GI(mod) (2.8 ± 0.8 vs 3.8 ± 1.3 mmol/l; P = 0.046). Basal and end of fast levels of insulin (basal insulin 71 ± 61 vs 20 ± 16 mU/l; P < 0.001; end of fast insulin 77 ± 51 vs 21 ± 20 mU/l; P < 0.001) and c-peptide (basal c-peptide 5.4 ± 2.4 vs 2.7 ± 1.6 μg/l; P = 0.004; end of fast c-peptide 5.3 ± 2.4 vs 2.5 ± 1.4 μg/l; P = 0.001) were significantly higher in GII/III(mod) than in GI(mod). No differences between the groups were observed when G(clas) criteria were applied. Additionally, close correlations were observed between insulin concentration, Ki-67 index and tumour size. CONCLUSION: This study shows an impact of histopathological tumour characteristics in patients suffering from an insulinoma on clinical presentation during a standardized 72-h fast. Lower initial blood glucose levels and higher concentrations of insulin and c-peptide are associated with worse tumour grading and larger tumour size.

6 Article Diagnosis and treatment of gastroenteropancreatic neuroendocrine tumors: current data on a prospectively collected, retrospectively analyzed clinical multicenter investigation. 2011

Niederle, Martin B / Niederle, Bruno. ·Section of Endocrine Surgery, Division of General Surgery, Department of Surgery, Medical University of Vienna, Währinger Gür, Austria. martin.niederle@meduniwien.ac.at ·Oncologist · Pubmed #21467149.

ABSTRACT: BACKGROUND: The aim of this prospectively collected, retrospectively analyzed clinical investigation was to describe "unmasked" clinical symptoms and methods of diagnosis, treatment, and short-term follow-up of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) diagnosed during 1 year in Austria. METHODS: In total, 277 patients with GEP-NETs were documented. All tumors were immunhistochemically defined according to recently summarized criteria (World Health Organization, European Neuroendocrine Tumour Society). A standardized questionnaire comprising 50 clinical and biochemical parameters (clinical symptoms, mode of diagnosis, treatment, follow-up) was completed by attending physicians. RESULTS: The most common initial symptoms were episodes of abdominal pain, diarrhea, weight loss, gastrointestinal bleeding, flushing, and bowel obstruction. Overall, 48.1% of tumors were diagnosed by endoscopy, 43.7% were diagnosed during surgery, 5% were diagnosed by fine-needle aspiration of the primary or metastases, and 2.5% were diagnosed during autopsy; 44.5% of tumors were not suspected clinically and were diagnosed incidentally during various surgical procedures. Overall, 18.7% of tumors were removed endoscopically and 67.6% were removed surgically; 13.7% of patients were followed without interventional treatment. Endoscopic or surgical intervention was curative in 81.4% of patients and palliative in 18.6% of patients. At the time of diagnosis, information on metastasis was available in 83.7% of patients with malignant NETs. Lymph node or distant metastases were documented in 74.7% of patients. In 19.3% of patients, 41 secondary tumors were documented, with 78.0% classified histologically as adenocarcinomas. CONCLUSION: This investigation summarizes the clinical presentation and current practice of management of GEP-NETs and thereby extends the understanding and clinical experience.

7 Article Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters. 2010

Niederle, Martin B / Hackl, Monika / Kaserer, Klaus / Niederle, Bruno. ·Division of General Surgery, Department of Surgery, Medical University of Vienna, Vienna, Austria. martin.niederle@meduniwien.ac.at ·Endocr Relat Cancer · Pubmed #20702725.

ABSTRACT: As incidence data on gastroenteropancreatic neuroendocrine tumours (GEP-NETs) have so far only been retrospectively obtained and based on inhomogeneous material, we conducted a prospective study in Austria collecting all newly diagnosed GEP-NETs during 1 year. Using the current WHO classification, the tumor, nodes, metastases (TNM) staging and Ki67 grading and the standard diagnostic procedure proposed by the European Neuroendocrine Tumor Society (ENETS), GEP-NETs from 285 patients (male: 148; female: 137) were recorded. The annual incidence rates were 2.51 per 100,000 inhabitants for men, 2.36 per 100,000 for women. The stomach (23%) was the main site, followed by appendix (21%), small intestine (15%) and rectum (14%). Patients with appendiceal tumours were significantly younger than patients with tumours in any other site. About 46.0% were classified as benign, 15.4% as uncertain, 31.9% as well differentiated malignant and 6.7% as poorly differentiated malignant. Patients with benign or uncertain tumours were significantly younger than patients with malignant tumours. Among the malignant tumours of the digestive tract, 1.49% arose from neuroendocrine cells. For malignant gastrointestinal NETs, the incidence was 0.80 per 100,000: 40.9% were ENETS stage I, 23.8% stage II, 11.6% stage III and 23.8% stage IV. The majority (59.7%) were grade 1, 31.2% grade 2 and 9.1% grade 3. NETs of the digestive tract are more common than previously reported; the majority show benign behaviour, are located in the stomach and are well differentiated. G3 tumours are very rare.

8 Article CXCL12/SDF-1 over-expression in human insulinomas and its biological relevance. 2009

Ilhan, Aysegul / Nabokikh, Anastasiya / Maj, Magdalena / Vidakovic, Melita / Nielsen, Jens H / Prikoszovich, Thomas / Niederle, Bruno / Base, Wolfgang / Luger, Anton / Wagner, Ludwig. ·Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria. ·Mol Cell Endocrinol · Pubmed #19013212.

ABSTRACT: This study was performed on the basis of previously obtained investigative gene array data concerning the over-expression of CXCL12/SDF-1 in human insulinomas versus human pancreatic islet preparations. The presence of CXCL12/SDF-1 was studied by RT-qPCR in human insulinomas (n=8) versus pancreatic islets (n=3), and was found to be significantly up-regulated in the former (p<0.012). The mRNA data were confirmed by immunostaining and confocal microscopy of human normal pancreatic islets, which showed the absence of CXCL12 protein and high expression in insulinoma tissue. Individual human insulinoma cells at cytospins stained positive for CXCL12 in the paranuclear region. These morphological data were extended by consecutive immunoblotting for cell-compartment-specific marker proteins of fractions obtained by sucrose gradient fractionation using Rin-5F insulinoma cells. CXCL12-containing fractions were positive for the membrane marker NSF but negative for SNAP-25 and appeared at a lighter density in the gradient than heavy insulin granules, suggesting packaging in specific granules different from insulin. In order to determine the biological relevance of the protein in insulinomas, we investigated the colony-forming potential of human CXCL12 stable-transfected rat Rin-5F insulinoma cells. These clones secreted human CXCL12 and contained 50-1000-fold higher copy numbers compared to its endogenous rat homologue. In colony-forming assays, these transfectant clones developed greater colony numbers, which were larger than wild-type and sham transfectants. To elucidate the mechanism of action, we identified a CXCL12 transfectant-specific increase in the pro-survival factor Mn-SOD, which is considered important for the inactivation of reactive oxygen species, thereby prolonging cell survival. These data demonstrate the importance of CXCL12 in the tumor biology of insulinoma.