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Gregory A Kaltsas, Jane Evanson, Alexandra Chrisoulidou, and Ashley B Grossman

). Only seven cases of intrasellar, two cases of suprasellar and four cases of parasellar non-malignant paragangliomas have been described ( Boari et al . 2006 ). Tumour location is more relevant than histology in assessing prognosis; the metastatic rate

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Alexandru Saveanu, Mihaela Muresan, Catherine De Micco, David Taieb, Anne-Laure Germanetti, Frederic Sebag, Jean-François Henry, Laurent Brunaud, Alain Enjalbert, Georges Weryha, and Anne Barlier

Introduction Pheochromocytomas (PCC) and paragangliomas (PGL) are neuroendocrine tumors derived from adrenal chromaffin cells and extra-adrenal paraganglia respectively ( Eisenhofer et al . 2008 ). These tumors cause variable secondary hypertension

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Nele Garbrecht, Martin Anlauf, Anja Schmitt, Tobias Henopp, Bence Sipos, Andreas Raffel, Claus F Eisenberger, Wolfram T Knoefel, Marianne Pavel, Christian Fottner, Thomas J Musholt, Anja Rinke, Rudolf Arnold, Uta Berndt, Ursula Plöckinger, Bertram Wiedenmann, Holger Moch, Philipp U Heitz, Paul Komminoth, Aurel Perren, and Günter Klöppel

paraganglioma causing biliary and pancreatic obstruction . International Journal of Gastrointestinal Cancer 29 93 – 98 . Chamberlain RS Blumgart LH 1999 Carcinoid tumors of the extrahepatic bile duct. A rare cause of malignant biliary obstruction . Cancer

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Luis G Perez-Rivas, Andrea Oßwald, Thomas Knösel, Kristin Lucia, Christian Schaaf, Michael Hristov, Julia Fazel, Thomas Kirchner, Felix Beuschlein, Martin Reincke, and Marily Theodoropoulou

low-grade mediastinal paraganglioma, 1 choriocarcinoma and 2 were classified as carcinoma of unknown primary (CUP) tumor. Tissue was available only from liver metastasis in 2 cases. Overall, metastases were detected in 10 patients (3 with SCLC, 1 with

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M Montani, A M Schmitt, S Schmid, T Locher, P Saremaslani, P U Heitz, P Komminoth, and A Perren

. 2001 ) have recently been described in familial paragangliomas and pheochromocytomas. Several reasons make these genes candidate tumor suppressors for MTC: (1) allelic loss of the short and long arm of chromosome 1, the localization of SDHB and SDHC

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Tobias Else

Introduction The last 15 years have brought significant advances in our understanding of the genetic basis of hereditary syndromes with a predisposition to pheochromocytoma (PC) and paraganglioma (PGL) (PCPGL) development. In the 1990s the genetic

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Carole Guerin, Pauline Romanet, David Taieb, Thierry Brue, André Lacroix, Frederic Sebag, Anne Barlier, and Frederic Castinetti

, pheochromocytoma; PGL, paraganglioma; PPGL, pheochromocytoma and paraganglioma. Pheochromocytoma in MEN2 and non-MEN2 familial forms PHEO are neuroendocrine tumors arising from adrenal medulla cells. Paragangliomas (PGL) originate form sympathetic

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Y M H Jonkers, S M H Claessen, A Perren, S Schmid, P Komminoth, A A Verhofstad, L J Hofland, R R de Krijger, P J Slootweg, F C S Ramaekers, and E-J M Speel

palliation, chemotherapy and anti-hypoglycemic medication ( Chun & Doherty 2001 , Komminoth et al. 2004 ). The only feature that separates benign from malignant disease is organ and/or lymph node infiltration or distant metastases ( Komminoth et al. 2004

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E Kim, E M Rath, V H M Tsang, A P Duff, B G Robinson, W B Church, D E Benn, T Dwight, and R J Clifton-Bligh

E Amar L Azriel S Bourdeau I Chabre O 2014 Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas . Human Molecular Genetics 23 2440 – 2446 . ( doi:10.1093/hmg/ddt639 ). Dahia PL

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Elena Rapizzi, Rossella Fucci, Elisa Giannoni, Letizia Canu, Susan Richter, Paolo Cirri, and Massimo Mannelli

only cancer cells but also non-malignant stromal cells, such as endothelial cells, fibroblasts, immune cells, and extracellular matrix, which together form the so-called tumor microenvironment. Over the past few years, it has become increasingly evident