Management of Pheochromocytomas and Paragangliomas.
Autor: | Passman JE; Department of Surgery, University of Pennsylvania Health System, 3400 Spruce Street, 4th Floor, Maloney Building, Philadelphia, PA 19104, USA. Electronic address: Jesse.passman@pennmedicine.upenn.edu., Wachtel H; Department of Surgery, University of Pennsylvania Health System, 3400 Spruce Street, 4th Floor, Maloney Building, Philadelphia, PA 19104, USA; Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA. |
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Jazyk: | angličtina |
Zdroj: | The Surgical clinics of North America [Surg Clin North Am] 2024 Aug; Vol. 104 (4), pp. 863-881. Date of Electronic Publication: 2024 Mar 23. |
DOI: | 10.1016/j.suc.2024.02.014 |
Abstrakt: | Pheochromocytomas and paragangliomas are distinctive neuroendocrine tumors which frequently produce excess catecholamines with resultant cardiovascular morbidity. These tumors have a strong genetic component, with up to 40% linked to hereditary pathogenic variants; therefore, germline genetic testing is recommended for all patients. Surgical resection offers the only potential cure in the case of localized disease. Given the potential for catecholaminergic crises, appropriate perioperative management is crucial, and all patients should undergo alpha-adrenergic blockade before resection. Therapeutic options for metastatic disease are limited and include surgical debulking, radiopharmaceutical therapies, and conventional chemotherapy. Competing Interests: Disclosure H. Wachtel received support from the National Institutes of Health, United States National Cancer Institute, United States Grant #K08 CA270385. (Copyright © 2024 Elsevier Inc. All rights reserved.) |
Databáze: | MEDLINE |
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