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Open access

Myrtille Fouché, Yves Bouffard, Mary-Charlotte Le Goff, Johanne Prothet, François Malavieille, Pierre Sagnard, Françoise Christin, Davy Hayi-Slayman, Arnaud Pasquer, Gilles Poncet, Thomas Walter, and Thomas Rimmelé

Introduction Small-bowel neuroendocrine tumours (SB-NETs) are rare secreting neoplasms. Hormones (serotonin, histamine, bradykinin, prostaglandins and chromogranin-A) released into the systemic circulation can lead to a carcinoid syndrome (CS

Open access

Magaly Zappa, Olivia Hentic, Marie-Pierre Vullierme, Matthieu Lagadec, Maxime Ronot, Philippe Ruszniewski, and Valérie Vilgrain

grouped as gastroenteropancreatic neuroendocrine tumours, or GEP-NETs. They are frequently metastasised at diagnosis, and the liver is the most common site of metastases ( 1 ). The presence of liver metastases is a significant negative prognostic factor

Open access

K G Samsom, L M van Veenendaal, G D Valk, M R Vriens, M E T Tesselaar, and J G van den Berg

Introduction Well-differentiated neuroendocrine tumours (NETs) represent a heterogeneous group of rare tumours, which have a relatively indolent disease course. Primary NETs can arise from neuroendocrine cells at various anatomic sites. They

Open access

E T Aristizabal Prada and C J Auernhammer

Introduction Neuroendocrine tumours (NETs) of the gastroenteropancreatic (GEP) system are often metastasized at the time of diagnosis and curative resection is not possible in all cases ( 1 , 2 ). Our knowledge on medical therapy of advanced

Open access

Ashley K Clift, Omar Faiz, Robert Goldin, John Martin, Harpreet Wasan, Marc-Olaf Liedke, Erik Schloericke, Anna Malczewska, Guido Rindi, Mark Kidd, Irvin M Modlin, and Andrea Frilling

Introduction Small bowel (SB) neuroendocrine tumours (NET) are accruing significant clinical attention due to their increasing incidence ( 1 , 2 ) in addition to recent advances in their molecular biology ( 3 , 4 , 5 , 6 ) and treatment

Open access

K E Lines, R P Vas Nunes, M Frost, C J Yates, M Stevenson, and R V Thakker

Introduction Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder, characterised by the combined occurrence of tumours of the parathyroid glands, and neuroendocrine tumours (NETs) of the pancreatic islets and anterior

Open access

Logan Mills, Panagiotis Drymousis, Yogesh Vashist, Christoph Burdelski, Andreas Prachalias, Parthi Srinivasan, Krishna Menon, Corina Cotoi, Saboor Khan, Judith Cave, Thomas Armstrong, Martin O Weickert, Jakob Izbicki, Joerg Schrader, Andreja Frilling, John K Ramage, and Raj Srirajaskanthan

Introduction Small pancreatic neuroendocrine tumours pose a management dilemma between surveillance and resection due to the uncertain natural history of these tumours. The case for surveillance in tumours ≤2 cm is supported by European

Open access

M S Elston, V B Crawford, M Swarbrick, M S Dray, M Head, and J V Conaglen

secondary to prostate neuroendocrine carcinoma ( 15 ). While somatostatin receptor scintigraphy can be helpful to identify neuroendocrine tumours such as bronchial carcinoids causing CS, given the likely aggressive underlying tumour in this setting, FDG

Open access

Erik Rösner, Daniel Kaemmerer, Elisa Neubauer, Jörg Sänger, and Amelie Lupp

Programmed death protein 1 (PD-1) and its ligand, PD-L1, have emerged as promising therapeutic targets for many types of cancer that overexpress PD-L1. However, data on PD-L1 expression levels in bronchopulmonary neuroendocrine neoplasms (BP-NEN) are limited and contradictory. In the present study, a total of 298 archived, formalin-fixed, paraffin-embedded BP-NEN samples from 97 patients diagnosed with typical carcinoid (TC), atypical carcinoid (AC), small cell lung cancer (SCLC), or large cell neuroendocrine carcinoma of the lung (LCNEC) were evaluated for PD-L1 expression by immunohistochemistry using the highly sensitive monoclonal anti-PD-L1 antibody 73-10. PD-L1 expression levels were semiquantitatively estimated by tumour grading. Of the 298 BP-NEN samples, 85% were positive for PD-L1 expression. PD-L1 immunostaining predominantly localized to the plasma membrane of both tumour cells and tumour-infiltrating immune cells. SCLC and LCNEC exhibited significantly higher PD-L1 expression levels than TC or AC. PD-L1 expression levels were also higher in patients with lymph node or distant metastases, in patients who smoked, and in patients who died during the follow-up period. Moreover, PD-L1 expression levels correlated positively with tumour grading, Ki-67 index and the expression of the chemokine receptor CXCR4 and negatively with the levels of somatostatin receptor 1 and chromogranin A. High tumour PD-L1 levels were associated with poor patient outcomes. In conclusion, PD-L1 expression is common in BP-NEN, increases with malignancy, and is associated with poor prognosis. Therefore, targeting the PD-1/PD-L1 axis could be a promising strategy for treating BP-NEN. PD-L1 may also represent a useful prognostic biomarker for this tumour entity.

Open access

Jean-Benoît Corcuff, Laurence Chardon, Ines El Hajji Ridah, and Julie Brossaud

Introduction Endocrine and neuroendocrine tumours diagnosis is based on the convergence of clinical, imaging and biological arguments. Tumour secretory markers are often hormones (or their metabolites) that are secreted by normal tissue as