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Oral Neurofibroma Clinical Presentation

  • Author: Indraneel Bhattacharyya, DDS, MSD; Chief Editor: William D James, MD  more...
 
Updated: Sep 04, 2015
 

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

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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Causes

The cause of these lesions is unknown; however, neurofibromatosis syndrome or the disseminated form is inherited as an autosomal dominant trait and may present with a variety of lesions, including a highly variable number of neurofibromas.

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Contributor Information and Disclosures
Author

Indraneel Bhattacharyya, DDS, MSD Professor, Department of Oral and Maxillofacial Diagnostic Sciences, Director of Oral and Maxillofacial Pathology Residency Program, University of Florida College of Dentistry

Indraneel Bhattacharyya, DDS, MSD is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Dental Association, International Association for Dental Research, International Association of Oral Pathologists, American Association for Dental Research

Disclosure: Nothing to disclose.

Coauthor(s)

Donald Cohen, DMD, MS Professor of Oral and Maxillofacial Pathology, Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida College of Dentistry

Donald Cohen, DMD, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Drore Eisen, MD, DDS Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati

Drore Eisen, MD, DDS is a member of the following medical societies: American Academy of Dermatology, American Academy of Oral Medicine, American Dental Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

References
  1. Depprich R, Singh DD, Reinecke P, Kübler NR, Handschel J. Solitary submucous neurofibroma of the mandible: review of the literature and report of a rare case. Head Face Med. Nov/2009. 13;5:24.:24. [Medline]. [Full Text].

  2. Marocchio LS, Oliveira DT, Pereira MC, Soares CT, Fleury RN. Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years. Clin Oral Investig. 2007 Jun. 11(2):165-9. [Medline].

  3. Johann AC, Caldeira PC, Souto GR, Freitas JB, Mesquita RA. Extra-osseous solitary hard palate neurofibroma. Braz J Otorhinolaryngol. 2008 Mar-Apr. 74(2):317. [Medline].

  4. Shimoyama T, Kato T, Nasu D, Kaneko T, Horie N, Ide F. Solitary neurofibroma of the oral mucosa: a previously undescribed variant of neurofibroma. J Oral Sci. 2002 Mar. 44(1):59-63. [Medline].

  5. Vivek N, Manikandhan R, James PC, Rajeev R. Solitary intraosseous neurofibroma of mandible. Indian J Dent Res. 2006 Jul-Sep. 17(3):135-8. [Medline].

  6. De Raedt T, Maertens O, Chmara M, et al. Somatic loss of wild type NF1 allele in neurofibromas: Comparison of NF1 microdeletion and non-microdeletion patients. Genes Chromosomes Cancer. 2006 Oct. 45(10):893-904. [Medline].

  7. Gomez-Oliveira G, Fernandez-Alba Luengo J. Martin-Sastre R, Patino-Seijas B, Lopez-Cedrun-Cembranos JL. Plexiform neurofibroma of the cheek mucosa. A case report. Med oral. May-Jul 2004. 9:263-7. [Medline]. [Full Text].

 
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Intrabony presentation of neurofibroma. Note the extensive bone destruction caused by the lesion.
Multiple neurofibromas on the tongue.
Solitary neurofibroma on the hard palate.
An 11-year-old girl with an asymptomatic raised lesion on the anterior mandibular gingiva.
Isolated palatal lesion in a 27-year-old African American woman.
 
 
 
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