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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

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Samira M Sadowski, Emanuel Christ, Benoit Bédat, Attila Kollár, Wolfram Karenovics, Aurel Perren, Frédéric Triponez and on behalf of the SwissNET registry

Introduction Neuroendocrine tumors of the lungs (LNET) represent approximately 30% of all NET ( 1 , 2 ) and account for 1–2% of all lung tumors. Lung NETs are classified into typical carcinoid (TC, low-grade tumors) and atypical carcinoid (AC

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Tiina Vesterinen, Teijo Kuopio, Maarit Ahtiainen, Aija Knuuttila, Harri Mustonen, Kaisa Salmenkivi, Johanna Arola and Caj Haglund

Introduction Pulmonary carcinoid (PC) tumors are low- and intermediate-grade neoplasms that are subdivided into typical carcinoid (TC) and atypical carcinoid (AC) based on the mitotic count and presence of necrosis ( 1 ). PCs belong to

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Irvin M Modlin, Harry Aslanian, Lisa Bodei, Ignat Drozdov and Mark Kidd

they comprise a heterogeneous group of cancers. Thus, tumor types range from histamine-secreting gastric ‘carcinoids’ to a mélange of pancreatic lesions (secreting glucagon, insulin, somatostatin, or vasoactive polypeptide – colloquially known as islet

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Merete Gedde-Dahl, Espen Thiis-Evensen, Andreas Myklebust Tjølsen, Kjerstin Skrede Mordal, Morten Vatn and Deidi S Bergestuen

with endocrine functions (1) . Small intestinal NETs often produce vasoactive substances that may result in the carcinoid syndrome, symptoms of which include flushing, diarrhea, carcinoid heart disease, and bronchospasm. The syndrome is almost

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Katherine Van Loon, Li Zhang, Jennifer Keiser, Cendy Carrasco, Katherine Glass, Maria-Teresa Ramirez, Sarah Bobiak, Eric K Nakakura, Alan P Venook, Manisha H Shah and Emily K Bergsland

(2) . Historically, bone metastases from NETs were considered to be extremely rare (3, 4) . As few as 50 case reports were identified in a recently published literature review on skeletal metastases from carcinoid tumors (5) . In a series of 145

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Roxanne C S van Adrichem, Aart Jan van der Lely, Martin Huisman, Piet Kramer, Richard A Feelders, Patric J D Delhanty and Wouter W de Herder

) and clinical parameters (age at diagnosis, sex, primary tumor location, carcinoid syndrome, ENETS TNM classification ( 32 , 33 ), Ki-67 proliferation index, grading, prior incomplete surgery) in NET patients. Subjects and methods NET

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Matilde Calanchini, Michael Tadman, Jesper Krogh, Andrea Fabbri, Ashley Grossman and Brian Shine

until they are large or have metastasised. However, up to 40% of patients, mainly with midgut carcinoids, present with features of the carcinoid syndrome (CS) ( 6 ), and undergo episodes of diarrhoea and flushing and occasionally asthma. Carcinoid heart

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Dong Cen, Hui Liu, Zhe Wan, Zhongjie Lin, Yanting Wang, Junjie Xu and Yuelong Liang

, also designated as carcinoid tumors in some systems, and poorly differentiated neuroendocrine carcinoma ( 5 ). NEN in the gallbladder is a relatively rare histological tumor type, accounting for 0.5% of all NEN cases ( 6 ). The most common histological

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Ashley K Clift, Oskar Kornasiewicz, Panagiotis Drymousis, Omar Faiz, Harpreet S Wasan, James M Kinross, Thomas Cecil and Andrea Frilling

Introduction Initially termed ‘goblet cell carcinoids’ ( 1 ), goblet cell carcinomas (GCC) are a rare sub-type of neoplasm arising from the appendix, accounting for less than 14% of all appendiceal tumours ( 2 ). Whilst an ‘intermediate