title: Clinical and Pathological Characteristics of Hypertensive and Normotensive Adrenal Pheochromocytomas.
abstract: BACKGROUND
  Pheochromocytoma/Paraganglioma (PPGL) present with an extremely variable clinical picture which ranges from dramatic, to mild, to silent, depending on tumor attitude to release catecholamines. Hypertension is the hallmark of these tumors but is not always present. Distinct differences of clinical manifestations exist in hypertensive pheochromocytomas (HPs) and normotensive pheochromocytomas (NPs), however the comparative analysis is lacking.
  
  
  METHODS
  The objective was to assess the clinical symptoms, hemodynamics, metabolism, radiological and histological features of patients with HPs and NPs. This study included 104 pheochromocytoma patients who were categorized into HPs (n=69) and NPs (n=35) groups. All clinical records were reviewed. Tumor samples were examined to determine the Adrenal Gland Scale Score and were available for measurement of gene transcriptions. Biochemical examinations of 95 subjects with primary hypertension (PH) were recorded for comparative study.
  
  
  RESULTS
  Patients with NPs showed lower proportion of clinical triad, inapparent metabolic disorders and lower urinary catecholamine levels than HPs, but higher than PH. Tumor weight positively correlated with 24 h urinary norepinephrine level in patients with HPs (P=0.028), and tumor diameter negatively correlated with phenylethanolamine-N-methyltransferase (PNMT) immunohistochemistry (P=0.011) in NPs but not in HPs. The Adrenal Gland Scale Score of NPs group was similar to that of HPs group. The positive percentage of epinephrine type (E-type) of catecholamine in HPs group was higher than that in the NPs. The transcript gene levels of PNMT, secretogranin II (SGII) and neuropeptide Y (NPY) from tissue samples were significantly lower in NPs than in HPs (PPNMT=0.038, PSGII=0.040, PNPY=0.032), while vesicular monoamine transporter 1 (VMAT1) had no difference between HPs and NPs (PVMAT1=0.053).
  
  
  CONCLUSIONS
  HPs and NPs have distinct differences in clinical, biochemical and pathological phenotypes, which are closely related with productions involved in tumor occurrence and development.
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: P
authors.initials: P
authors.lastname: Li
authors.affiliation: Department of Urology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: W
authors.initials: W
authors.lastname: Gan
authors.affiliation: Department of Urology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: X
authors.initials: X
authors.lastname: Zhao
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: S
authors.initials: S
authors.lastname: Shen
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: W
authors.initials: W
authors.lastname: Feng
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: Q
authors.initials: Q
authors.lastname: Xu
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Bi
authors.affiliation: Department of Urology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: H
authors.initials: H
authors.lastname: Guo
authors.affiliation: Department of Endocrinology, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, China.
authors.forename: D
authors.initials: D
authors.lastname: Zhu
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000503:physiopathology; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006973:Hypertension / Q000000981:diagnostic imaging / Q000209:etiology / Q000378:metabolism* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D010673:Pheochromocytoma / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000503:physiopathology; D012189:Retrospective Studies