Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas
Por:
Garcia-Carbonero R, Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Aristegui M, Bella-Cueto M, Ferrer-Albiach C, Hanzu F
Publicada:
1 oct 2021
Ahead of Print:
1 may 2021
Resumen:
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.
Filiaciones:
Garcia-Carbonero R:
UCM, Hosp Univ 12 Octubre, Inst Invest Sanitaria Hosp 12 Octubre Imas12, Dept Med Oncol,CNIO,CIBERONC, Avda Cordoba Km 5-4, Madrid 28041, Spain
Teresa F:
Hosp Clin San Carlos, Dept Radiol, Madrid, Spain
Mercader-Cidoncha E:
Hosp Gen Univ Gregorio Maranon, Gen & Digest Surg Dept, Endocrine & Metab Surg Unit, Madrid, Spain
Mitjavila-Casanovas M:
Hosp Univ Puerta Hierro, Dept Nucl Med, Majadahonda, Spain
Grp Trabajo Endocrino SEMNIM, Madrid, Spain
Robledo M:
Spanish Natl Canc Res Ctr, Hereditary Endocrine Canc Grp, Madrid, Spain
Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid, Spain
Tena I:
Med Sci Innovat Res MedSIR CORP, Dept Sci, Ridgewood, NJ USA
Hosp Prov, Dept Med Oncol, Castellon de La Plana, Spain
Alvarez-Escola C:
Hosp Univ La Paz, Endocrinol & Nutr Dept, Neuroendocrinol Unit, Madrid, Spain
Aristegui M:
Hosp Gen Univ Gregorio Maranon, ENT Dept, Madrid, Spain
Bella-Cueto M:
Univ Autonoma Barcelona, Dept Pathol, Inst Invest & Innovacio Parc Tauli I3PT, Hosp Univ Parc Tauli, Sabadell, Spain
Ferrer-Albiach C:
Hosp Prov Castellon, Dept Radiat Oncol, Castellon de La Plana, Spain
Hanzu F:
Univ Barcelona, Hosp Clin Barcelona, Endocrinol & Nutr Dept, IDIBAPS, Barcelona, Spain
Green Published, hybrid
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