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

• R. Garcia Carbonero ^[1] ; F. Matute Teresa ^[2] ; E. Mercader Cidoncha ^[3] ; M. Mitjavila Casanovas ^[4] ; M. Robledo ^[8] ; I. Tena ^[9] ; C. Alvarez Escola ^[5] ; M. Arístegui ^[3] ; M. R. Bella Cueto ^[6] ; C. Ferrer Albiach ^[10] ; F. A. Hanzu ^[7]
  1. [1] Hospital Universitario 12 de Octubre

     Hospital Universitario 12 de Octubre

     Madrid, España

     
  2. [2] Hospital Clínico San Carlos de Madrid

     Hospital Clínico San Carlos de Madrid

     Madrid, España

     
  3. [3] Hospital General Universitario Gregorio Marañón

     Hospital General Universitario Gregorio Marañón

     Madrid, España

     
  4. [4] Hospital Universitario Puerta de Hierro

     Hospital Universitario Puerta de Hierro

     Madrid, España

     
  5. [5] Hospital Universitario La Paz

     Hospital Universitario La Paz

     Madrid, España

     
  6. [6] Universitat Autònoma de Barcelona

     Universitat Autònoma de Barcelona

     Barcelona, España

     
  7. [7] Universitat de Barcelona

     Universitat de Barcelona

     Barcelona, España

     
  8. [8] Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center, Madrid, Spain
  9. [9] Medical Oncology Department, Hospital Provincial, Castellon, Spain
  10. [10] adiation Oncology Department, Hospital Provincial Castellón, Castellón, Spain

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• Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 23, Nº. 10, 2021, págs. 1995-2019
• Idioma: inglés
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• 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; anat- omy, 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.