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Woo-Jae Park, Ori Brenner, Aviram Kogot-Levin, Ann Saada, Alfred H Merrill Jr, Yael Pewzner-Jung, and Anthony H Futerman

, in all cases with hyperplasia, the architecture of the adrenal gland was well-preserved and without significant cortical atrophy ( Fig. 6 D and E). Paragangliomas were not identified. None of the histologic changes (i.e. PCC or hyperplasia) were

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L Fishbein, S Ben-Maimon, S Keefe, K Cengel, D A Pryma, A Loaiza-Bonilla, D L Fraker, K L Nathanson, and D L Cohen

Dear Editor, Pheochromocytomas and paragangliomas (PCCs/PGLs) are tumors in the adrenal medulla and extra-adrenal ganglia, respectively. About a quarter are metastatic, often with a long latency period. Some PCC/PGLs are defined as clinically

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Jenny Welander, Peter Söderkvist, and Oliver Gimm

% of all the pheochromocytomas or paragangliomas are thought to be associated with germline SDHx mutations ( Gill et al . 2010 , Welander et al . 2011 ), no single SDHx gene is affected in more than 10% of the cases at the most. Genome

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E E King, Y Qin, R A Toledo, A Luo, E Ball, F R Faucz, K A Janeway, C A Stratakis, G E Tomlinson, and P L M Dahia

Dear Editor, Germline mutations of the tumor suppressor gene TMEM127 occur in the neural-crest-derived tumors, pheochromocytomas and paragangliomas ( Qin et al . 2010 , Yao et al . 2010 , Neumann et al . 2011 ), and have also been detected in

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Hartmut P H Neumann

pheochromocytomas are present. Figure 1 Pheochromocytomas and paragangliomas (arrows in A–E). (A) Bilateral adrenal tumors in multiple endocrine neoplasia type 2. Image: courtesy Martin K Walz, Huyssens Hospital, Essen, MD. (B) Extra-adrenal retroperitoneal

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V Häyry, K Salmenkivi, J Arola, P Heikkilä, C Haglund, and H Sariola

adrenal medulla. Extra-adrenal phaeochromocytomas arising from the paraganglion system are called paragangliomas. They represent ∼15–20% of all phaeochromocytomas ( Lenders et al . 2005 ). Paragangliomas seem to be more aggressive and associated with

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Adrian F Daly, Emilie Castermans, Lindsey Oudijk, Mirtha A Guitelman, Pablo Beckers, Iulia Potorac, Sebastian J C M M Neggers, Nathalie Sacre, Aart-Jan van der Lely, Vincent Bours, Wouter W de Herder, and Albert Beckers

papillary thyroid cancer. She underwent total thyroidectomy, with clear lymph node and neck dissection. Given the history of tumors in three endocrine glands she underwent genetic testing for pheochromocytoma-paraganglioma, pituitary and MEN syndrome genes

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

, respectively. Collectively, these changes are thought to drive tumorigenesis in the multiple organ systems that demonstrate increased incidence of neoplasia in individuals harboring germline SDHx mutations, which include paragangliomas

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Graeme Eisenhofer, Stefan R Bornstein, Frederieke M Brouwers, Nai-Kong V Cheung, Patricia L Dahia, Ronald R de Krijger, Thomas J Giordano, Lloyd A Greene, David S Goldstein, Hendrik Lehnert, William M Manger, John M Maris, Hartmut P H Neumann, Karel Pacak, Barry L Shulkin, David I Smith, Arthur S Tischler, and William F Young Jr

of the RET gene, neurofibromatosis type 1 (NF1) due to mutations of the NF gene, and familial paraganglioma and/or pheochromocytoma syndromes caused by mutations of genes for members of the succinate dehydrogenase family ( SDHB , SDHC , and SDHD

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Ying Ni, Spencer Seballos, Shireen Ganapathi, Danielle Gurin, Benjamin Fletcher, Joanne Ngeow, Rebecca Nagy, Richard T Kloos, Matthew D Ringel, Thomas LaFramboise, and Charis Eng

neurodegenerative disease ( Bourgeron et al . 1995 , Parfait et al . 2000 ). In contrast, germline heterozygous mutations in the genes encoding the SDH subunits result in hereditary pheochromocytoma–paraganglioma syndrome ( Baysal et al . 2000 , Neumann et al