title: The value of plasma markers for the clinical behaviour of phaeochromocytomas.
abstract: OBJECTIVE
  Phaeochromocytomas (PCCs) are widely known for their clinical unpredictability. This study intends to define predictive plasma markers for their variable postoperative behaviour. Furthermore, the diagnostic accuracy of these plasma tests was determined.
  
  
  METHODS
  A retrospective correlative study was performed in a series of 83 operated and four autopsied patients in order to correlate preoperative catecholamine (CAT) levels of 103 PCCs with their clinical behaviour. In a subset of cases, chromogranin-A (Chr-A) and enzymes/precursors of the CAT biosynthesis were studied for their predictive value.
  
  
  RESULTS
  Basal CAT levels were elevated in 81/87 instances (sensitivity: 93%). Four of six cases with normal measurements showed only medullary hyperplasia. Larger PCCs, particularly those showing necrosis, capsular and vascular invasion, secreted higher CAT levels. Bilateral, hereditary tumours were less productive than their unilateral counterparts. Extra-adrenal PCCs secreted significantly lower levels of epinephrine (EPI) than intra-adrenal tumours. Fourteen patients developed metastases. According to Kaplan-Meier estimations, patients with higher levels of dopamine, norepinephrine (NE) and aromatic l-amino acid decarboxylase as well as lower ratios of EPI/EPI+NE, had significantly shorter metastases-free intervals. Existence of preoperative hypertension, left ventricular hypertrophy and measured blood pressures showed significant positive relationships with CAT levels, but not with Chr-A.
  
  
  CONCLUSIONS
  These data showed that plasma CAT measurement is a sensitive method in the diagnostic work-up of PCCs. Those tumours producing normal levels are commonly small and asymptomatic. Furthermore, certain secretion patterns are indicative of the presence of metastases as well as the size and site of sporadic and syndrome-related PCCs.
authors.affiliation: Department of Surgery, Erasmus University Hospital and MCRZ location Clara, Rotterdam, The Netherlands. harstE@mcrz.nl
authors.forename: E
authors.initials: E
authors.lastname: van der Harst
authors.forename: W W
authors.initials: WW
authors.lastname: de Herder
authors.forename: R R
authors.initials: RR
authors.lastname: de Krijger
authors.forename: H A
authors.initials: HA
authors.lastname: Bruining
authors.forename: H J
authors.initials: HJ
authors.lastname: Bonjer
authors.forename: S W J
authors.initials: SW
authors.lastname: Lamberts
authors.forename: A H
authors.initials: AH
authors.lastname: van den Meiracker
authors.forename: T H
authors.initials: TH
authors.lastname: Stijnen
authors.forename: F
authors.initials: F
authors.lastname: Boomsma
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000097:blood* / Q000175:diagnosis* / Q000601:surgery; D000328:Adult; D000368:Aged; D001142:Aromatic-L-Amino-Acid Decarboxylases / Q000097:blood; D015415:Biomarkers; D001794:Blood Pressure; D002395:Catecholamines / Q000097:blood / Q000378:metabolism; D002648:Child; D053379:Chromogranin A; D002864:Chromogranins / Q000097:blood; D018572:Disease-Free Survival; D004299:Dopamine beta-Hydroxylase / Q000097:blood; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000097:blood* / Q000175:diagnosis* / Q000601:surgery; D011183:Postoperative Complications / Q000097:blood / Q000175:diagnosis; D011237:Predictive Value of Tests; D011300:Preoperative Care; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D002395:Catecholamines; D053379:Chromogranin A; D002864:Chromogranins; D004299:Dopamine beta-Hydroxylase; D001142:Aromatic-L-Amino-Acid Decarboxylases