Abstract
Paragangliomas and pheochromocytomas (PPGLs) are rare neuroendocrine tumors originating from paraganglionic tissue in many sites of the body. Most PPGLs are characterized by nonaggressive behavior but all of them have the potential to metastasize. PPGLs represent a great diagnostic dilemma as it is difficult to recognize tumors that are likely to be metastasizing; criteria of malignancy can be found both in benign and metastatic forms. This review aims to analyze the current knowledge of the nature of metastasizing PPGLs paying particular attention to head and neck paragangliomas (HNPGLs). Potential predictors of the malignancy risk for PPGLs were summarized and discussed. These data may also help in the development of diagnostic and prognostic strategies, as well as in the identification of novel potential therapeutic targets for patients with PPGLs.
Highlights
Paragangliomas and pheochromocytomas (PPGLs) are rare neuroendocrine tumors formed from paraganglionic tissue
This review summarized the current findings on the potential markers for distinguishing between metastasizing and benign tumors, as well as on the prediction of aggressive behavior of PPGLs, especially of those localized in the head and neck region
CPGL, carotid paraganglioma, MEPGL, middle ear paraganglioma, VPGL, vagal paraganglioma. * Metastatic cases are characterized by a 10-year earlier age of diagnosis. ** The ratio is higher in populations living at high altitudes under hypoxic conditions. *** Incidence of multiple paragangliomas increases to 30–40% in patients with a positive family history
Summary
Paragangliomas and pheochromocytomas (PPGLs) are rare neuroendocrine tumors formed from paraganglionic tissue. Tumors developing from paraganglia outside the adrenal gland are termed paragangliomas (PGLs). Parasympathetic paraganglia include supracardiac paraganglia, paraganglia of the carotid body, middle ear, and larynx, as well as paraganglia distributed along the vagus nerve and several other smaller paraganglia [3]. These paraganglia are common throughout the body, but most are found in the head and neck area [4]. The most common sites for head and neck paragangliomas (HNPGLs) include the carotid body followed by middle ear and vagal glomus [1]. CPGL, carotid paraganglioma, MEPGL, middle ear paraganglioma, VPGL, vagal paraganglioma. * Metastatic cases are characterized by a 10-year earlier age of diagnosis. ** The ratio is higher in populations living at high altitudes under hypoxic conditions. *** Incidence of multiple paragangliomas increases to 30–40% in patients with a positive family history
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