Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this art...

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Detalles Bibliográficos
Autores: García-Carbonero, Rocío|||0000-0002-3342-397X, Matute Teresa, F.|||0000-0001-8691-5997, Mercader-Cidoncha, E.|||0000-0002-1836-0165, Mitjavila-Casanovas, M.|||0000-0001-6030-4266, Robledo, Mercedes|||0000-0001-6256-5902, Tena, I.|||0000-0003-4199-5799, Álvarez-Escolá, Cristina|||0000-0002-4684-1652, Arístegui, M.|||0000-0003-0680-2568, Bella-Cueto, Maria Rosa|||0000-0002-5144-0490, Ferrer-Albiach, C.|||0000-0001-9122-7646, Hanzu, Felicia A.|||0000-0002-2399-4023
Tipo de recurso: artículo
Fecha de publicación:2021
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:249566
Acceso en línea:https://ddd.uab.cat/record/249566
https://dx.doi.org/urn:doi:10.1007/s12094-021-02622-9
Access Level:acceso abierto
Palabra clave:Pheochromocytoma
Paraganglioma
Diagnosis
Treatment
Genetic counseling
Multidisciplinary
Guidelines
Descripción
Sumario:Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.

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