TY - CHAP T1 - Paraganglioma BT - StatPearls A1 - Ikram,Asad, AU - Rehman,Anis, Y1 - 2021/01// PY - 2019/11/22/pubmed PY - 2019/11/22/medline PY - 2019/11/22/entrez N2 - Paragangliomas are rare, catecholamine (norepinephrine) secreting neuroendocrine tumors commonly located in pre-aortic and paravertebral sympathetic plexus or skull base. Head-and-neck paragangliomas in the jugular foramen, ear, or carotid body are less differentiated tumors. They secrete norepinephrine as compared to more differentiated intraabdominal adrenal medulla tumors like neuroblastoma and pheochromocytoma (also called intra-adrenal paragangliomas, which primarily secrete epinephrine).[1] Glomus jugulare and carotid body paragangliomas comprise 80% of paragangliomas in the head-and-neck. Functionally, pheochromocytomas/paragangliomas are highly vascular and may be either parasympathetic or sympathetic. The parasympathetic tumors are usually asymptomatic and inactive, located mostly in the skull base in the distribution of IX and X cranial nerves. In contrast, sympathetic lesions are highly active and symptomatic and mainly located in the abdomen and pelvic regions. They are more functional and hypersecretory (norepinephrine) than the skull base paragangliomas. Paragangliomas are commonly a single unilateral tumor, but 1% vs. 20 to 80% may be multiple in sporadic vs. familial types, respectively.[2] They are usually benign tumors, and a small percentage may become malignant and metastasize. Hence, an early identification leading to complete surgical resection is often curative and carries a favorable prognosis. Detecting distant metastases is the only reliable way to assess the biological aggressiveness of paragangliomas. They are mostly sporadic, but 30 to 40% are familial, and some of those may be associated with genetic syndromes like succinate dehydrogenase (SDH) / B-mutations, Carney-stratakis dyad, neurofibromatosis type 1 (NF1), Von Hippel Lindau (VHL), and multiple endocrine neoplasia types 2A and 2B (MEN2).[3] Surgical biopsy of the lesion is the gold standard to confirm the diagnosis, but it does not differentiate between pheochromocytomas and paragangliomas. Clinical correlation is usually enough for the diagnosis aided by imaging and pathology findings of the lesion. PB - StatPearls Publishing CY - Treasure Island (FL) UR - https://www.unboundmedicine.com/medline/citation/31751024/StatPearls:_Paraganglioma L2 - https://www.ncbi.nlm.nih.gov/books/NBK549834 DB - PRIME DP - Unbound Medicine ER -