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Matthew H Kulke, Thomas O'Dorisio, Alexandria Phan, Emily Bergsland, Linda Law, Phillip Banks, Joel Freiman, Kenny Frazier, Jessica Jackson, James C Yao, Larry Kvols, Pablo Lapuerta, Brian Zambrowicz, Douglas Fleming and Arthur Sands

Introduction Carcinoid syndrome is characterized by watery diarrhea, episodic flushing, bronchoconstriction, and eventually, the development of right-sided valvular heart disease. The symptoms of carcinoid syndrome have been attributed, in part, to

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Johannes Hofland, Aura D Herrera-Martínez, Wouter T Zandee and Wouter W de Herder

of the cardiac valves ( van der Lely & de Herder 2005 ). The main mediator of carcinoid-related sequelae, particularly diarrhea and fibrosis, is thought to be the amine derivative serotonin (5-hydroxytryptamine). Other co-secreted peptide hormones and

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C A Koch, F M Brouwers, K Rosenblatt, K D Burman, M M Davis, A O Vortmeyer and K Pacak

Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. There are presently no markers that can reliably distinguish benign and malignant neuroendocrine tumors. Here we describe a 63-year-old woman who developed sudden chest pain and hypertension combined with increased stool frequency. An incidental adrenal mass 5 cm in size with a bright signal on T2-weighted magnetic resonance imaging was discovered. Biochemical evaluation and (131)I-metaiodobenzylguanidine (MIBG) scintigraphy were negative. Histopathological examination revealed a mature adrenal GN. Neuroblastoma, the immature form of a GN, is known for deletions on chromosomal locus 1p36, and adrenal tumors frequently show allele loss on 17p. To further elucidate the histo- and pathogenesis of adrenal GN, we performed loss of heterozygosity studies on chromosomal loci 1p34-36 and 17p13 (the p53 gene locus) after careful microdissection of tumor and normal tissue. We did not detect allelic losses at these loci with the informative polymorphic markers used, suggesting that these loci are not involved in tumorigenesis. In addition, immunohistochemical investigation of the GN was positive for vasoactive intestinal peptide, a hormone commonly expressed in ganglion cells. We suggest that in our patient with an adrenal GN, the combination of biochemical, scintigraphic, molecular, immunohistochemical, and histopathological findings are all consistent with the benign morphology of this tumor.

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Larry K Kvols, Kjell E Oberg, Thomas M O'Dorisio, Pharis Mohideen, Wouter W de Herder, Rudolf Arnold, Ke Hu, Yilong Zhang, Gareth Hughes, Lowell Anthony and Bertram Wiedenmann

these amines and peptide hormones, especially serotonin and substance P, is responsible for the principal symptoms of diarrhea and flushing associated with carcinoid syndrome. Surgery is essential in the management of metastatic NETs and can be curative

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Marianne Pavel, David J Gross, Marta Benavent, Petros Perros, Raj Srirajaskanthan, Richard R P Warner, Matthew H Kulke, Lowell B Anthony, Pamela L Kunz, Dieter Hörsch, Martin O Weickert, Pablo Lapuerta, Wenjun Jiang, Kenneth Kassler-Taub, Suman Wason, Rosanna Fleming, Douglas Fleming and Rocio Garcia-Carbonero

Introduction Carcinoid syndrome (CS) is a disorder that develops in up to 20% of patients with neuroendocrine tumors (NETs) and is characterized by severe diarrhea, flushing, abdominal pain, and eventually cardiac valvular complications, which

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Francis Worden, Martin Fassnacht, Yuankai Shi, Tatiana Hadjieva, Françoise Bonichon, Ming Gao, Laura Fugazzola, Yuichi Ando, Yasuhisa Hasegawa, Do Joon Park, Young Kee Shong, Johannes W A Smit, John Chung, Christian Kappeler, Gerold Meinhardt, Martin Schlumberger and Marcia S Brose

, diarrhea, fatigue, and weight loss ( Escudier et al . 2007 , Llovet et al . 2008 , Cheng et al . 2009 , Brose et al . 2014 a ). DECISION was a large Phase 3 randomized, placebo-controlled trial in patients with locally advanced or metastatic

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Jennifer A Chan, David P Ryan, Andrew X Zhu, Thomas A Abrams, Brian M Wolpin, Paige Malinowski, Eileen M Regan, Charles S Fuchs and Matthew H Kulke

-refractory carcinoid syndrome ( Kvols et al . 2006 , Petersenn et al . 2010 ). In these studies, doses of pasireotide ranged from 200 to 1200 μg s.c. twice daily. Adverse events were generally mild, and included hyperglycemia, nausea, diarrhea, and abdominal pain. A

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Chen-Tian Shen, Zhong-Ling Qiu and Quan-Yong Luo

(Q-Y L) assessed all the discrepant items. To evaluate the toxicity of sorafenib, the authors calculated the number of events of the following AEs reported in the safety profile section: hand–foot syndrome, diarrhea, fatigue, rash, weight loss, and

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O Hentic, P Hammel, A Couvelard, V Rebours, M Zappa, M Palazzo, F Maire, G Goujon, A Gillet, P Lévy and P Ruszniewski

of ≥grade 3 diarrhea or ≥grade 3 neutropenia/thrombopenia. Tumor response rate was assessed by CT scan at 3-month intervals according to Response Evaluation in Solid Tumors (RECIST) criteria ( Therasse et al . 2000 ). The best response was considered

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Wouter T Zandee, Kimberly Kamp, Roxanne C van Adrichem, Richard A Feelders and Wouter W de Herder

-syndromic, NET. Small intestinal NETs almost exclusively secrete serotonin, and when metastasized causing the carcinoid syndrome with symptoms of flushing, diarrhea and carcinoid heart disease. Pancreatic NETs show a much larger variation in hormonal secretion