Head and neck paragangliomas: Recent advances in translational and clinical research and guidelines for patient care.

Autor: Richter S; Institute for Clinical Chemistry and Laboratory Medicine, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany. Electronic address: susan.richter@ukdd.de., Constantinescu G; Department of Internal Medicine III, University Clinic Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany., Fancello G; Department of Otology and Skull Base Surgery, Gruppo Otologico, 29121 Piacenza, Italy., Paties CT; Pathology Unit, San Raffaele Scientific Institute, IRCCS, 20132 Milan, Italy., Mariani-Costantini R; Center for Advanced Studies and Technology (CAST), G. d'Annunzio University, Via Luigi Polacchi 11, 66100 Chieti, Italy. Electronic address: renato.mariani@unich.it., Sanna M; Department of Otology and Skull Base Surgery, Gruppo Otologico, 29121 Piacenza, Italy.
Jazyk: angličtina
Zdroj: Best practice & research. Clinical endocrinology & metabolism [Best Pract Res Clin Endocrinol Metab] 2024 Sep 11, pp. 101951. Date of Electronic Publication: 2024 Sep 11.
DOI: 10.1016/j.beem.2024.101951
Abstrakt: Head and neck paragangliomas (HNPGLs), rare neuroendocrine tumors that mainly arise from parasympathetic ganglia along the cranial nerves, are challenging due to anatomic origin, tendency to aggressive neurovascular and skull base infiltration, unpredictable metastatic potential, radio-chemoresistance, and risk of multiplicity. Symptoms range from mild to life threatening depending on location/size, but rarely relate to catecholamine excess. Risk factors include female sex and pathogenic germline variants in genes affecting hypoxia signaling (foremost succinate dehydrogenase genes). Diagnostic work-up relies on imaging, measurements of plasma free metanephrines/methoxytyramine, genetic testing, and pathology/immunohistochemistry. Management is tailored to patient/tumor characteristics and encompasses wait-scan, upfront surgery, debulking surgery, and radiotherapy. Presurgical embolization is recommended, except for small tympanic and tympanomasoid tumors. Presurgical stenting is required for internal carotid artery involvement, and two-stage surgery for intradural extension. Current treatments for metastatic/inoperable HNPGL are non-curative, and long-term follow-up should be recommended for all patients to monitor local recurrence and new tumors.
(Copyright © 2024. Published by Elsevier Ltd.)
Databáze: MEDLINE
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