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Misu Lee, Ninelia Minaskan, Tobias Wiedemann, Martin Irmler, Johannes Beckers, Behrooz H Yousefi, Georgios Kaissis, Rickmer Braren, Iina Laitinen, and Natalia S Pellegata

resistance to rapamycin in human cancer cells . Molecular Pharmacology 54 815 – 824 . Huang H Abraham J Hung E Averbuch S Merino M Steinberg SM Pacak K Fojo T 2008 Treatment of malignant pheochromocytoma/paraganglioma

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Aguirre A de Cubas, L Javier Leandro-García, Francesca Schiavi, Veronika Mancikova, Iñaki Comino-Méndez, Lucía Inglada-Pérez, Manuel Perez-Martinez, Nuria Ibarz, Pilar Ximénez-Embún, Elena López-Jiménez, Agnieszka Maliszewska, Rocío Letón, Álvaro Gómez Graña, Carmen Bernal, Cristina Álvarez-Escolá, Cristina Rodríguez-Antona, Giuseppe Opocher, Javier Muñoz, Diego Megias, Alberto Cascón, and Mercedes Robledo

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors of neural crest origin. Up to an estimated 60% of PCCs/PGLs are associated with germline or somatic mutations in one many susceptibility genes ( RET

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Margo Dona, Selma Waaijers, Susan Richter, Graeme Eisenhofer, Jeroen Korving, Sarah M Kamel, Jeroen Bakkers, Elena Rapizzi, Richard J Rodenburg, Jan Zethof, Marnix Gorissen, Gert Flik, Peter M T Deen, and Henri J L M Timmers

of a heterozygous SDH mutation are at risk of developing pheochromocytomas and paragangliomas (PPGLs), although usually a second hit in the form of a somatic mutation in the unaffected SDHB allele is required for the development of PPGLs. PPGLs

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Alberto Delgado Verdugo, Joakim Crona, Lee Starker, Peter Stålberg, Göran Åkerström, Gunnar Westin, Per Hellman, and Peyman Björklund

N Karimi M Lee JJ Backdahl M Ekstrom TJ Larsson C 2008 Global and regional CpG methylation in pheochromocytomas and abdominal paragangliomas: association to malignant behavior . Clinical Cancer Research 14 2551 – 2559 . ( doi:10.1158/1078-0432.CCR

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Gregory Kaltsas, Ioannis I Androulakis, Wouter W de Herder, and Ashley B Grossman

Paraganglioma and carcinoid Malignant hypertension ↑Pro-renin, aldosterone (+) Radiology Hypokalemia alkalosis (+) Octreoscan Gut hormones Ghrelin Carcinoid and PICT Unknown clinical phenotype ↑Ghrelin (+) Radiology (+) Octreoscan Gastrin PICT Zollinger

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Georgios K Dimitriadis, Anna Angelousi, Martin O Weickert, Harpal S Randeva, Gregory Kaltsas, and Ashley Grossman

hormone by tumour cells in vitro PNS can develop during different phases of the evolution of the neoplastic process, with an estimated prevalence of approximately 8% among all malignant neoplasms ( Baijens et al. 2006 ). In some cases, PNS

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Martin Ullrich, Josephine Liers, Mirko Peitzsch, Anja Feldmann, Ralf Bergmann, Ulrich Sommer, Susan Richter, Stefan R Bornstein, Michael Bachmann, Graeme Eisenhofer, Christian G Ziegler, and Jens Pietzsch

Jeunemaitre X Gimenez-Roqueplo AP , 2007 Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . ( https://doi.org/10

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Dorota Dworakowska and Ashley B Grossman

carcinomas (PCs) are a rare clinical entity that represent only 0.1–0.2% of all pituitary tumours. Oncological malignant potential is defined by the ability to metastasise (including craniospinal and/or systemic metastases). Early identification of pituitary

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Thomas J Giordano

.13–2.51 Francis (2013) Adrenal cortical carcinoma 0.9 0.2–14.0 Zheng et al . (2016) Pheochromocytoma/paraganglioma Low Unknown Fishbein et al . (2017) Small cell lung carcinoma 8.62 Unknown George et al . (2015) Merkel

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Pei-Pei Xu, Su Zeng, Xiao-Tian Xia, Zi-Heng Ye, Mei-Fang Li, Ming-Yun Chen, Tian Xia, Jing-Jing Xu, Qiong Jiao, Liang Liu, Lian-Xi Li, and Ming-Gao Guo

that FNAB is unable to distinguish malignant from benign thyroid follicular lesions ( Mazzanti et al. 2004 , Cooper et al. 2009 , Layfield et al. 2009 , Olson et al. 2012 ). Therefore, the search for specific markers of FTC remains one of