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James F Powers, Brent Cochran, James D Baleja, Hadley D Sikes, Xue Zhang, Inna Lomakin, Troy Langford, Kassi Taylor Stein and Arthur S Tischler

Introduction SDH-deficient gastrointestinal stromal tumors (GISTs) are rare but sometimes lethal tumors that can occur in patients with paraganglioma (PGL) syndromes. These tumors do not harbor the KIT or PDGFRA mutations typical of

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Eva Szarek, Evan R Ball, Alessio Imperiale, Maria Tsokos, Fabio R Faucz, Alessio Giubellino, François-Marie Moussallieh, Izzie-Jacques Namer, Mones S Abu-Asab, Karel Pacak, David Taïeb, J Aidan Carney and Constantine A Stratakis

Introduction Carney triad (CTr) is a syndrome that describes the association of paragangliomas (PGLs) with gastrointestinal (GI) stromal tumors (GISTs) and pulmonary chondromas; other lesions, including pheochromocytomas, esophageal leiomyomas, and

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Priya Gopie, Lin Mei, Anthony C Faber, Steven R Grossman, Steven C Smith and Sosipatros A Boikos

Introduction Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) system ( Boikos & Stratakis 2014 ), with more than 5000 newly diagnosed cases yearly in the United States ( Barnett et al

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Florian Haller, Evgeny A Moskalev, Fabio R Faucz, Sarah Barthelmeß, Stefan Wiemann, Matthias Bieg, Guillaume Assie, Jerome Bertherat, Inga-Marie Schaefer, Claudia Otto, Eleanor Rattenberry, Eamonn R Maher, Philipp Ströbel, Martin Werner, J Aidan Carney, Arndt Hartmann, Constantine A Stratakis and Abbas Agaimy

Introduction Carney triad (CT) is a rare condition with synchronous or metachronous occurrence in a patient of three tumor entities, namely gastric gastrointestinal stromal tumor (GIST), paraganglioma (PGL), and pulmonary chondroma ( Carney et al

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Per Bümming, Ola Nilsson, Håkan Ahlman, Anna Welbencer, Mattias K Andersson, Katarina Sjölund and Bengt Nilsson

Introduction Gastrointestinal stromal tumors (GIST) are thought to originate from stem cells that differentiate toward the interstitial cells of Cajal (ICC; Kindblom et al. 1998 , Sircar et al. 1999 , Lee et al. 2001 ). The

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Sara Ekeblad, Bengt Nilsson, Margareta Halin Lejonklou, Térèse Johansson, Peter Stålberg, Ola Nilsson, Håkan Ahlman and Britt Skogseid

Introduction Gastrointestinal stromal tumors (GISTs) originate from pacemaker cells (the interstitial cells of Cajal) and therefore exhibit a phenotype with expression of CD34 and the KIT tyrosine kinase receptor ( Kindblom et al

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Catherine J Williams, Nicholas Mitsiades, Elias Sozopoulos, Alex Hsi, Alicja Wolk, Artemissia-Phoebe Nifli, Sofia Tseleni-Balafouta and Christos S Mantzoros

. 2005 ). However, it remains unknown whether adiponectin receptors are expressed in the gastrointestinal tract, and expression of AdipoR1 and AdipoR2 has not been previously studied in colorectal carcinomas or gastrointestinal stromal tumors (GISTs

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V H M Tsang, T Dwight, D E Benn, G Y Meyer-Rochow, A J Gill, M Sywak, S Sidhu, D Veivers, C M Sue, B G Robinson, R J Clifton-Bligh and N R Parker

accumulation which in turn stabilises HIFα and therefore activation of a hypoxic gene signature ( Favier & Gimenez-Roqueplo 2010 ). Gastrointestinal stromal tumours (GISTs) arise from the pacemaker cells of the gastrointestinal tract, and 85–90% of GISTs are

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Thomas G Papathomas, Lindsey Oudijk, Ellen C Zwarthoff, Edward Post, Floor A Duijkers, Max M van Noesel, Leo J Hofland, Patrick J Pollard, Eamonn R Maher, David F Restuccia, Richard A Feelders, Gaston J H Franssen, Henri J Timmers, Stefan Sleijfer, Wouter W de Herder, Ronald R de Krijger, Winand N M Dinjens and Esther Korpershoek

, PGLs, and gastrointestinal stromal tumors (GISTs) was explored. Subjects and methods Tumor tissue samples and cell lines A total of 253 formalin-fixed and paraffin-embedded (FFPE) tumors were selected, including 38 ACC samples (Erasmus MC, Rotterdam

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Adrian F Daly and Albert Beckers

stromal tumor (GIST; at the time gastric ‘leiomyosarcoma’), paraganglioma, and pulmonary chondroma. While it is traditional to use the term GIST in describing the gastric lesions in Carney triad, they are probably better described as ‘gastric stromal