title: GROWTH RATE OF PARAGANGLIOMAS RELATED TO GERMLINE MUTATIONS OF THE SDHX GENES.
abstract: OBJECTIVE
  The purpose was to determine the growth rate of succinate dehydrogenase subunit (SDHx) gene-related paragangliomas based on computed tomography (CT) measurements.
  
  
  METHODS
  Twenty-seven patients with SDHx mutations who underwent subsequent CT examinations were enrolled in the study. Tumors were classified as head and neck (HNP), thoracic, or abdominal/pelvic paragangliomas (PGLs). The percentage volume increase and volume doubling time were estimated.
  
  
  RESULTS
  We analyzed 56 PGLs (21 with SDHD, 6 with SDHB mutations) in 27 patients (16 men, 11 women; mean age 37.7 years). The estimated median of the follow-up was 23 months. Twenty-two (39.3%) PGLs were located in the abdomen, 8 (14.3%) in the thorax, and 26 (46.4%) in the head and neck region. The median volume growth rate was estimated at 10.4% per year (interquartile range [IQR]: -1.3; 36.3). The volume doubling time was estimated as 7.01 (2.24;+∞) years. By tumor site, the estimated medians of the annual volume growth rates were 13.6% (IQR:0.8 -30.4) for HNP, -6.06% (IQR: -1.79;47.32) for thoracic PGLs, and 10.5% (IQR: -2.2;44.6) for abdominal PGLs. The volume doubling time was 5.44 years (2.61; 87.0) for HNP, 11.8 years (1.79;+∞) for thoracic PGLs, and 6.94 years (1,88;+∞) for abdominal PGLs. There was no significant difference in the volume growth rate according to tumor location or initial size (P>.7 and P = .07, respectively) or gene mutation type (SDHB vs. SDHD, P>.8).
  
  
  CONCLUSIONS
  PGLs related to SDHx mutations are slowly growing tumors. There were no correlations between tumor location, growth rate or initial size over a 23-month follow-up period.
  
  
  BACKGROUND
  CT = computed tomography HNP = head and neck paraganglioma IQR = interquartile range PGL = paraganglioma PPGL = pheochromocytoma and paraganglioma SDH = succinate dehydrogenase.
authors.forename: Ilona
authors.initials: I
authors.lastname: Michałowska
authors.forename: Jarosław B
authors.initials: JB
authors.lastname: Ćwikła
authors.forename: Wojciech
authors.initials: W
authors.lastname: Michalski
authors.forename: Lucjan S
authors.initials: LS
authors.lastname: Wyrwicz
authors.forename: Aleksander
authors.initials: A
authors.lastname: Prejbisz
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Szperl
authors.forename: Dawid
authors.initials: D
authors.lastname: Nieć
authors.forename: Hartmut P H
authors.initials: HP
authors.lastname: Neumann
authors.forename: Andrzej
authors.initials: A
authors.lastname: Januszewicz
authors.forename: Mariola
authors.initials: M
authors.lastname: Pęczkowska
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000235:genetics; D000328:Adult; D018450:Disease Progression; D005260:Female; D018095:Germ-Line Mutation; D006801:Humans; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology*; D010673:Pheochromocytoma / Q000235:genetics; D013385:Succinate Dehydrogenase / Q000235:genetics*; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: C491545:SDHD protein, human; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase