Oral Neurofibroma Clinical Presentation

Updated: Jun 19, 2018
  • Author: Indraneel Bhattacharyya, DDS, MSD; Chief Editor: Jeff Burgess, DDS, MSD  more...
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Presentation

History

Patients usually present with an uninflamed, slowly enlarging, asymptomatic lesion that varies greatly in size from tiny nodules to large pendulous masses. The lesion is rarely painful; however, patients may experience pain if the lesion is secondarily traumatized due to its location, eg, on the tongue or on the hard palate.

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Physical Examination

Oral neurofibromas usually present as submucosal, nontender, discrete masses that range in size from a few millimeters to several centimeters (see images below). The lesions are typically pedunculated or sessile, usually painless, but occasionally pain or paresthesia is reported due to nerve compression. Typically, lesions are less than 2 cm in greatest diameter. Larger lesions of up to 8 centimeters are usually seen in syndromic cases.

Multiple neurofibromas on the tongue. Multiple neurofibromas on the tongue.
Solitary neurofibroma on the hard palate. Solitary neurofibroma on the hard palate.
An 11-year-old girl with an asymptomatic raised le An 11-year-old girl with an asymptomatic raised lesion on the anterior mandibular gingiva.
Isolated palatal lesion in a 27-year-old African A Isolated palatal lesion in a 27-year-old African American woman.

The tumors tend to grow slowly, and patients are usually asymptomatic.

Manifestations of neurofibromatosis specific to the oral cavity include enlarged fungiform papillae on the dorsum of the tongue and diffuse enlargement of the gingiva.

In patients with mandibular involvement, enlargement of the inferior alveolar canal in the mandible and a flaring of the inferior alveolar foramen (the so-called blunderbuss foramen) have been reported.

Oral manifestations may be seen in as many as 70% of patients with neurofibromatosis. Involvement of the trigeminal nerve may cause facial pain or paresthesia. Neurofibromatosis of the skin may present as multiple nodules or as a single pendulous mass.

In patients with neurofibromatosis, extensive destruction of alveolar bone, mimicking periodontal bone loss, has been reported. This may be confused with routine periodontitis or other systemic disease. Owing to the potential systemic and genetic implications, the diagnosis of oral neurofibroma requires referral to the appropriate medical specialist to rule out the association with neurofibromatosis.

The tongue, the buccal mucosa, and the vestibular areas are the most common sites of presentation.

Rare cases of diffuse unilateral enlargement of gingiva have been reported in association with neurofibromatosis. In a recent case report, an 8-year-old child presented with gingival enlargement around all teeth of the right side of both jaws. This may resemble drug-induced gingival enlargement or gingival fibromatosis.

Occasional cases of neurofibroma located centrally within the jaw have been reported. These intraosseous lesions may exhibit large sizes with a considerable expansion potential (see image below).

Multiple neurofibromas on the tongue. Multiple neurofibromas on the tongue.
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