title: Glomus tumors in individuals with neurofibromatosis type 1.
abstract: BACKGROUND
  Glomus tumors have recently been reported in individuals with the neurofibromatosis type 1 (NF1) cancer disposition syndrome. We compare the clinical and molecular features of these painful hamartomas in a series of sporadic and NF1-associated cases.
  
  
  OBJECTIVE
  We sought to evaluate the association of NF1 with glomus tumors and to compare NF1-associated glomus tumors with sporadic glomus tumors.
  
  
  METHODS
  We conducted a retrospective cohort study of all individuals with a histopathologic diagnosis of glomus tumor at a large tertiary care center from January 1998 to January 2013. Charts were reviewed for a coexisting diagnosis of NF1.
  
  
  RESULTS
  A total of 42 glomus tumors were identified in 34 individuals. Twelve (28.6%) were found in 6 patients with NF1. In 28 individuals with 30 sporadic tumors, there was no coexisting medical condition. Although multifocal tumors (16.7%) and tumor recurrence (33.3%) were more common in association with NF1, these trends did not reach statistical significance. NF1-associated glomus tumors exhibited no neurofibromin immunoreactivity, whereas their sporadic counterparts retained neurofibromin expression.
  
  
  CONCLUSIONS
  The retrospective design resulted in incomplete data capture.
  
  
  CONCLUSIONS
  Detection of glomus tumors should raise suspicion for a concurrent diagnosis of NF1.
authors.affiliation: Division of Dermatology, Department of Internal Medicine, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Monique G
authors.initials: MG
authors.lastname: Kumar
authors.affiliation: Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Ryan J
authors.initials: RJ
authors.lastname: Emnett
authors.affiliation: Division of Dermatology, Department of Internal Medicine, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Susan J
authors.initials: SJ
authors.lastname: Bayliss
authors.affiliation: Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri. Electronic address: gutmannd@neuro.wustl.edu.
authors.forename: David H
authors.initials: DH
authors.lastname: Gutmann
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005918:Glomus Tumor / Q000150:complications* / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000378:metabolism; D025542:Neurofibromin 1 / Q000378:metabolism; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D025542:Neurofibromin 1
keywords: glomus tumor; neurofibromatosis; neurofibromatosis type 1; neurofibromin; tumor disposition syndrome