title: Malignant pheochromocytoma-paraganglioma: pathogenesis, TNM staging, and current clinical trials.
abstract: OBJECTIVE
  Pheochromocytomas and paragangliomas (PPGs) are rare neuroendocrine tumors. Over the last 15 years, substantial progress has been made toward understanding the clinical aspects and molecular origins of this disease. Nevertheless, predicting and managing malignancy remains the biggest challenge in clinical practice. The natural history of patients with malignant PPGs has not yet been described, and their prognosis varies. Currently, the diagnosis of malignant PPGs relies on the presence of metastases, by which time the disease is usually advanced. Better understanding of the clinical and molecular characteristics of patients with malignant PPGs has spurred several prospective clinical trials.
  
  
  RESULTS
  Several molecular targeted therapies, a novel radiopharmaceutical medication that targets the catecholamine transporter, and immunotherapy are under evaluation for the treatment of patients with malignant PPGs. Furthermore, the identification of clinical predictors of malignancy and survival has led to the first TNM staging classification for PPGs.
  
  
  CONCLUSIONS
  Prospective clinical trials are providing patients with therapeutic options beyond systemic chemotherapy. The knowledge derived from these trials and from the evaluation of the TNM staging in clinical practice will help to clarify how to most effectively treat malignant PPGs.
authors.affiliation: aDepartment of Endocrinology, Hospital Universitario San Vicente Fundacion-Universidad de Antioquia, Medellín, Colombia bDepartment of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Roman-Gonzalez
authors.forename: Camilo
authors.initials: C
authors.lastname: Jimenez
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D058990:Molecular Targeted Therapy / Q000379:methods; D009367:Neoplasm Staging; D010235:Paraganglioma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D010673:Pheochromocytoma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D019275:Radiopharmaceuticals / Q000627:therapeutic use
chemical_list: D019275:Radiopharmaceuticals