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Introduction Phaeochromocytomas/paragangliomas (PHAEO/PG) are chromaffin cell tumours, which can occur sporadically or as part of other hereditary syndromes including multiple endocrine neoplasia 2 (MEN-2), Von Hippel–Lindau syndrome (VHL) and
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Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumours arising from chromaffin cells in adrenal medulla and autonomous ganglia. A majority of these tumours have a low proliferation and seldom metastasize. The understanding
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Introduction Pheochromocytomas (PCC)–paragangliomas (PGL) (PPGL) are rare tumors of neural crest origin with malignant potential. The prevalence of metastasis ranges from 2–13% in PCC to 2.4–50% in PGL ( 1 , 2 ). In patients with unresectable
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Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus an der Technische Universität Dresden, Dresden, Germany
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Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus an der Technische Universität Dresden, Dresden, Germany
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Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland
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Introduction Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors that derive from adrenomedullary tissue (pheochromocytomas) in about 85% of cases and from extra-adrenal chromaffin tissue (paragangliomas) in the remainder
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Introduction Pheochromocytoma and paraganglioma (PPGL) are tumors composed of chromaffin cells, which commonly produce one or more catecholamines: epinephrine, norepinephrine and dopamine. These tumors are rarely silent, and they are
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Introduction Composite pheochromocytoma/paraganglioma (CP) is a rare neoplasm consisting of pheochromocytoma (PCC) or paraganglioma (PGL) combined with developmentally related neurogenic tumor ( 1 ). Its neurogenic component is various
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CINTESIS, Center for Health Technology and Services Research, University of Porto, Porto, Portugal
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Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Porto, Portugal
Department of Clinical Pathology, Centro Hospitalar Universitário de São João (CHUSJ), Porto, Portugal
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Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Porto, Portugal
Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal
Department of Endocrinology, Centro Hospitalar Universitário de São João (CHUSJ), Porto, Portugal
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neoplasms, such as hemangioblastomas and pheochromocytomas. Pheochromocytomas and paragangliomas (PPGLs), arising either through germline VHL mutations in a syndromic setting or via somatic VHL mutations sporadically, associate with the pseudo
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particularly true in the case of pheochromocytomas and paragangliomas (PPGL) ( 1 ). These neuroendocrine tumors produce symptoms due to direct tumor effects and the synthesis and release of bioactive amines, neurotransmitters, and hormones; about 20 new
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Faculty of Life Sciences and Medicine, King’s College London, London, UK
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Faculty of Life Sciences and Medicine, King’s College London, London, UK
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Introduction Phaeochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumours arising from neural crest tissue. Phaeochromocytoma (PCC) arises from the adrenal medulla and paragangliomas (PGLs) from autonomic ganglia, usually arising
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Introduction Pheochromocytomas/paragangliomas (PCC/PGLs) are tumors, arose from neural crest-derived chromaffin cells, produce and secrete catecholamines ( 1 , 2 , 3 ). PCCs are tumors of the adrenal medulla and PGLs originate from