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Michael Solarski, Fabio Rotondo, William D Foulkes, John R Priest, Luis V Syro, Henriett Butz, Michael D Cusimano, and Kalman Kovacs

function . Nature Genetics 41 365 – 370 . ( https://doi.org/10.1038/ng.317 ) 10.1038/ng.317 19219043 Mertens WC Grignon DJ Romano W 1993 Malignant paraganglioma with skeletal metastases and spinal cord compression: response and palliation with

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Patricia L M Dahia, Roderick Clifton-Bligh, Anne-Paule Gimenez-Roqueplo, Mercedes Robledo, and Camilo Jimenez

dacarbazine for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis . Clinical Endocrinology 81 642 – 651 . ( https://doi.org/10.1111/cen.12542 ) O’Kane GM Ezzat S Joshua AM Bourdeau I Leibowitz-Amit R Olney HJ

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Joakim Crona, Angela Lamarca, Suman Ghosal, Staffan Welin, Britt Skogseid, and Karel Pacak

22 302 – 314 . van Hulsteijn LT Dekkers OM Hes FJ Smit JW Corssmit EP 2012 Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis . Journal of Medical Genetics 25 25 . ( https

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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

JJ 2009 Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma . Journal of Clinical Endocrinology and Metabolism 94 5 – 9 . ( doi:10.1210/jc.2008-1836 ) Lee M Waser B Reubi JC

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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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Anna Karpathakis, Martyn Caplin, and Christina Thirlwell

in the treatment of malignant paraganglioma/pheochromocytoma . Journal of Clinical Endocrinology and Metabolism 94 5 – 9 . doi:10.1210/jc.2008-1836 . Karar J Maity A 2011 PI3K/AKT/mTOR pathway in angiogenesis . Frontiers in Molecular

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Sophie Moog, Charlotte Lussey-Lepoutre, and Judith Favier

G van Hulsteijn LT Dekkers OM Corssmit EPM 2014 Chemotherapy with cyclophosphamide, vincristine and dacarbazine for malignant paraganglioma and pheochromocytoma: systematic review and meta-analysis . Clinical Endocrinology 81 642 – 651

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Asterios Karagiannis, Dimitri P Mikhailidis, Vasilios G Athyros, and Faidon Harsoulis

[ 111 In]octreotide uptake in begnign and malignant pheochromocytomas . Journal of Clinical Endocrinology and Metabolism 86 685 – 693 . Havekes B Corssmit EP Jansen JC van der Mey AG Vriends AH Romijn JA 2007 Malignant paragangliomas

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Alexandra Chrisoulidou, Gregory Kaltsas, Ioannis Ilias, and Ashley B Grossman

(mammalian target of rapamycin) inhibitor everolimus (RAD001, Novartis) has been shown to have some efficacy in neuroendocrine tumours generally, although our experience with this drug in two patients with malignant paragangliomas was not very positive. Due

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Camilo Jimenez, Sasan Fazeli, and Alejandro Román-Gonzalez

of Oncology 22 2661 – 2666 . ( https://doi.org/10.1093/annonc/mdr011 ) Joshua AM Ezzat S Asa SL Evans A Broom R Freeman M Knox JJ 2009 Rationale and evidence for sunitinib in the treatment of malignant paraganglioma