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Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine
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The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

by Sylvia L. Asa 1,2,3, Shereen Ezzat 2,4,5 and Ozgur Mete 1,2,3,*
1
Department of Pathology, University Health Network, Toronto, ON M5G 2C4, Canada
2
Endocrine Oncology Site, Princess Margaret Cancer Center, Toronto, ON M5G 2MG, Canada
3
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON M5S 1A8, Canada
4
Department of Medicine, Division of Endocrinology, University Health Network, Toronto, ON M5G 2C4, Canada
5
Department of Medicine, University of Toronto, Toronto, ON M5S 1A8, Canada
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2018, 7(9), 280; https://doi.org/10.3390/jcm7090280
Received: 30 July 2018 / Revised: 6 September 2018 / Accepted: 10 September 2018 / Published: 13 September 2018
(This article belongs to the Special Issue Pheochromocytoma and Paraganglioma: Research Update)
Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the head and neck, being most frequent in the carotid body, followed by jugulotympanic paraganglia, vagal nerve and ganglion nodosum, as well as laryngeal paraganglia. Abdominal sites include the well-known urinary bladder tumors that originate in the Organ of Zuckerkandl. However, other unusual sites of origin include peri-adrenal, para-aortic, inter-aortocaval, and paracaval retroperitoneal sites, as well as tumors in organs where they may not be expected in the differential diagnosis of neuroendocrine neoplasms, such as thyroid, parathyroid, pituitary, gut, pancreas, liver, mesentery, lung, heart and mediastinum. The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. Secondly, the genetic implications are different, since paragangliomas/pheochromocytomas have the highest rate of germline susceptibility at almost 40%. Finally, the characterization of metastatic disease is unique in these highly syndromic lesions. In this review, we summarize updated concepts by outlining the spectrum of anatomic locations of paragangliomas, the importance of morphology in establishing the correct diagnosis, the clinical implications for management, and the impact of genetics on the distinction between multifocal primary tumors compared with malignant disease. View Full-Text
Keywords: pheochromocytoma; paraganglioma; genetic susceptibility; SDHB; metastatic paraganglioma; catecholamines pheochromocytoma; paraganglioma; genetic susceptibility; SDHB; metastatic paraganglioma; catecholamines
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Asa, S.L.; Ezzat, S.; Mete, O. The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations. J. Clin. Med. 2018, 7, 280.

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