title: Comparison of clinical and imaging features in succinate dehydrogenase-positive versus sporadic paragangliomas.
abstract: BACKGROUND
  Limited data exist concerning the clinical and imaging features that distinguish sporadic from familial paragangliomas (PGLs).
  
  
  METHODS
  Clinical, genetic (succinate dehydrogenase [SDHB] vs no SDHx), and imaging (computed tomography [CT], magnetic resonance imaging, (18)F-fluoro-deoxy-glucose positron emission tomography [(18)F-FDG-PET]) features obtained during a decade in 124 PGL patients were studied. Data were analyzed by Fisher's exact test or Wilcoxon rank-sum test.
  
  
  RESULTS
  Mean age at diagnosis was younger in the SDHB-positive (SDHB+) group compared with the sporadic (no SDHx) group (28 vs 39 years, respectively, P < .001). Rate of supradiaphragmatic neoplasms were greater in the SDHB+ group (16.7 vs 4.7%, P = .11). Metastasis rates were greater in the SDHB+ group (78.9 vs 48.3%, P < .001), as was the existence of metastases or multiple PGLs at presentation (38.5 vs 16.7%, P < .05). Tumor volumes >250 mL were exclusively observed in SDHB+ patients (P < .05). On CT, SDHB+ tumors were more enhanced (P < .05). On (18)F-FDG-PET, SDHB+ tumors' had greater mean standard uptake values (12.3 vs 8.0, P < .05).
  
  
  CONCLUSIONS
  Clinically young age, large tumor volume, greater rate of metastatic and multifocal PGLs, greater SUV values on (18)F-FDG-PET, and increased CT enhancement are observed in SDHB+ PGLs. These findings may warrant genetic screening. Because SDHB+ patients demonstrate more supradiaphragmatic lesions, whole-body imaging may be of particular value in these patients.
authors.affiliation: Center for Interventional Oncology, Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD20892, USA. VenkatesanA@cc.nih.gov
authors.forename: Aradhana M
authors.initials: AM
authors.lastname: Venkatesan
authors.forename: Hari
authors.initials: H
authors.lastname: Trivedi
authors.forename: Karen T
authors.initials: KT
authors.lastname: Adams
authors.forename: Electron
authors.initials: E
authors.lastname: Kebebew
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.forename: Marybeth S
authors.initials: MS
authors.lastname: Hughes
mesh_terms: D000328:Adult; D000367:Age Factors; D014408:Biomarkers, Tumor / Q000235:genetics*; D005260:Female; D018095:Germ-Line Mutation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009362:Neoplasm Metastasis; D010235:Paraganglioma / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics / Q000473:pathology; D049268:Positron-Emission Tomography; D012008:Recurrence; D012189:Retrospective Studies; D013385:Succinate Dehydrogenase / Q000235:genetics*; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor; D013385:Succinate Dehydrogenase