Oral manifestations in patients with neurofibromatosis type-1: a comprehensive literature review

Crit Rev Oncol Hematol. 2014 Aug;91(2):123-9. doi: 10.1016/j.critrevonc.2014.02.007. Epub 2014 Mar 12.


Oral health status is jeopardized in patients with neurofibromatosis (NF) type-1 (NF-1). The aim of the present study was to comprehensively review the oral manifestations in NF-1 patients. PubMed/Medline and Google-Scholar databases were explored using different keywords. Reviews, commentaries, letters to Editor and articles published in languages other than English were excluded.

Results: Neurofibromas of oral and perioral soft tissues with subsequent periodontitis, impacted and supernumerary teeth, enlarged alveolar process with dental spacing, morphological changes in teeth and class III molar relationship have been reported in NF-1 patients. Plexiform neurofibromas were reported both in oral soft tissue, maxilla and mandible with evidence of malignant transformation in some cases. Facial skeletal abnormalities, including enlargement of mandibular foramen, increased dimensions of the coronoid and sigmoid notches and notching of the posterior border of the mandible have also been reported. Association between dental caries and NF-1 remains unclear.

Keywords: Dental; Inflammation; Neurofibromatosis type-I; Oral and periodontal.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Dental Caries / complications
  • Dental Caries / pathology
  • Gingiva / pathology
  • Humans
  • Mandible / pathology
  • Maxilla / pathology
  • Mouth / pathology*
  • Mouth Neoplasms / complications*
  • Mouth Neoplasms / epidemiology
  • Mouth Neoplasms / pathology*
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / epidemiology
  • Neurofibromatosis 1 / pathology*
  • Periodontitis / complications
  • Periodontitis / pathology
  • Tooth / pathology