title: Are patients with hormonally functional phaeochromocytoma and paraganglioma initially receiving a proper adrenoceptor blockade? A retrospective cohort study.
abstract: Pharmacological treatment is mandatory in patients with hormonally functional phaeochromocytoma and paraganglioma (PHAEO/PGL). We evaluated if patients initially diagnosed with hormonally functional PHAEO/PGL by various medical subspecialties received proper adrenoceptor blockade, and analysed factors predicting the prescription of adequate treatment.
  
  
  
  In a retrospective cohort study, we reviewed data from patients initially diagnosed with hormonally functional PHAEO/PGL outside the National Institutes of Health and Cedars-Sinai Medical Center, who were referred to these institutions between January 2001 and April 2015. Logistic regression was used to assess factors associated with proper adrenoceptor blockade.
  
  
  
  A total of 381 patients were included. Adequate pharmacological treatment was prescribed to 69·3%, of which 93·1% received α-adrenoceptor blockers. Regarding patients who were inappropriately treated, 53% did not receive any medication. Independent predictors of the prescription of a proper blockade were the diagnosis by endocrinologists [odds ratio (OR) 4·14; 95% confidence interval (CI), 2·51-6·85; P < 0·001], the presence of high blood pressure (OR 5·94; 95% CI, 3·11-11·33; P < 0·001) and the evidence of metastasis (OR 5·96; 95% CI, 1·93-18·46; P = 0·002).
  
  
  
  Although most patients received adequate pharmacological treatment, almost one-third were either not treated or received inappropriate medications. The diagnosis by endocrinologists, the presence of high blood pressure and the evidence of metastatic disease were identified as independent predictors of a proper blockade. These results highlight the need to educate physicians about the importance of starting adequate adrenoceptor blockade in all patients with hormonally functional PHAEO/PGL.
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.|Department of Endocrinology and Diabetology, Hospital Garcia de Orta, Almada, Portugal.
authors.forename: Henrique Vara
authors.initials: HV
authors.lastname: Luiz
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.|Section of Endocrinology and Metabolism, University of Santo Tomas Hospital, España, Manila, Philippines.
authors.forename: Mary Jane
authors.initials: MJ
authors.lastname: Tanchee
authors.affiliation: Clinical Neuroendocrinology Branch, National Institute of Mental Health, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Maria G
authors.initials: MG
authors.lastname: Pavlatou
authors.affiliation: Division of Endocrinology and Carcinoid and Neuroendocrine Tumour Center, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
authors.forename: Run
authors.initials: R
authors.lastname: Yu
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Joan
authors.initials: J
authors.lastname: Nambuba
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Katherine
authors.initials: K
authors.lastname: Wolf
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Tamara
authors.initials: T
authors.lastname: Prodanov
authors.affiliation: Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Wesley
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Karen
authors.initials: K
authors.lastname: Adams
authors.affiliation: Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Tito
authors.initials: T
authors.lastname: Fojo
authors.affiliation: Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000188:drug therapy*; D018674:Adrenergic Antagonists / Q000627:therapeutic use*; D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006973:Hypertension; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010235:Paraganglioma / Q000188:drug therapy*; D010673:Pheochromocytoma / Q000188:drug therapy*; D017410:Practice Guidelines as Topic / Q000592:standards*; D011941:Receptors, Adrenergic; D012189:Retrospective Studies; D014481:United States; D055815:Young Adult
chemical_list: D018674:Adrenergic Antagonists; D000959:Antihypertensive Agents; D011941:Receptors, Adrenergic