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CASE REPORT Table of Contents   
Year : 2006  |  Volume : 17  |  Issue : 3  |  Page : 135-8
Solitary intraosseous neurofibroma of mandible.

Department of Oral and Maxillofacial Surgery, Meenakshi Ammal Dental College and Hospital, Chennai, India

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Solitary intraosseous neurofibroma is a rare benign non-odontogenic tumor. Although neurofibromas occur predominantly as a feature of neurofibromatosis affecting the soft tissue, a few cases of solitary intraosseous neurofibromas of the jaw have been reported. We herewith report a case of solitary intraosseous neurofibroma of mandible in a middle-aged woman with a discussion on its clinical, radiological, and histopathological presentation along with review of cases.

Keywords: Neurofibroma, intraosseous lesion, neurofibromatosis, S100

How to cite this article:
Vivek N, Manikandhan R, James P C, Rajeev R. Solitary intraosseous neurofibroma of mandible. Indian J Dent Res 2006;17:135

How to cite this URL:
Vivek N, Manikandhan R, James P C, Rajeev R. Solitary intraosseous neurofibroma of mandible. Indian J Dent Res [serial online] 2006 [cited 2018 Jun 19];17:135. Available from:

   Introduction Top

Neurofibromatosis (NF) is an autosomal dominant disease affecting the neural crest cells that give rise to the ectodermal and mesodermal derivatives. The disease affects 1 in every 3000 and it has the highest mutation rates amongst genetic diseases [1]. Intraorally the various sites of occurrence in the soft tissue are tongue, floor of mouth, buccal mucosa, edentulous alveolar ridge, gingiva and pharyngo-maxillary spaces have been reported [1]. The reported cases of solitary intraosseous neurofibroma are few in the literatures [2],[3]. With the advent ofnewer analytical techniques, the incidence of this condition has increased from previously reported 4% to 72% [4].

   Case report Top

A39-year-old female patient reported with complaint of sudden and spontaneous loss of teeth with severe recession of the gingiva of the mandibular anterior/premolar region. The spontaneous exfoliation of lower right lateral incisor, canine and first premolar occurred within a period of six months. Patient experienced tingling sensation of the lower lip continuously during the entire above-mentioned period. Hermedical and familial history were noncontributory [Figure 1,2].

Physical examination showed a well-nourished woman with no apparent distress. As there was limited response to pinprick and temperature sensation in the lower lip on the affected side, a complete cranial nerve assessment was performed, with no positive findings.

Clinically, there was asymmetry of the face with obvious extraoral swelling towards the right lower border of the mandible. Normal jaw movements could be elicited and the right submandibular lymph nodes were palpable and tender.

Intraoral examination revealed missing 42, 43 and 44 with resorbed alveolar ridge. A moderate mobility of right lower second premolar was noticed. The region was non-tender and no discharge was seen. There was no sign of any ulcer in the lesion site with normal mucosa overlying it. Soft tssue in the other areas of the oral cavity appeared normal.

Orthopantomograph revealed a relatively well-circumscribed radiolucent area in the symphyseal and right parasymphyseal region extending from 45 to 31 regions [Figure - 3]. The continuation of the mandibular canal was lost anterior to the 2nd premolar area and was merging into the lesion beyond that.

After an informed consent, an incisional biopsy was performed under local anesthesia and sent for histopathological examination.


On microscopical examination, spindle shaped cells with wavy and tapered nuclei were seen arranged in fascicles in a haphazard manner. Few chronic inflammatory cells were also seen. The lack of encapsulation and focal areas of positivity for S-100 protein by using immuno histochemistry was highly suggestive of an intraosseous neurofibroma [Figure 4-8].

Surgical Management

It was decided to surgically remove the lesion and the patient was informed about the excision of the nerve and its consequences. An intraoral excision was done under general anesthesia after providing adequate clearance of about 6 mm on either side to prevent recurrence. Segmental resection of mandible included removal of 2nd premolar and left central and lateral incisors. The lesion was found to originate from inferior alveolar nerve and was removed as one piece. Even though immediate reconstruction was suggested with autologous bone graft or transport distraction but the patient refused this option, therefore a definitive reconstruction was planned secondarily. In order to bridge the defect a reconstruction plate was used. One year of follow up revealed no recurrence and the patient remained asymptomatic [Figure - 8].

   Discussion Top

Most nerve sheath tumors are true neoplasms. Neurofibromas of the oral cavity are usually solitary. Even though the solitary intraosseous neural tumors are rare, the occurrence of neurofibromas, nemilemmomas and ganglionemomas are common [5].

NF is one of the four neurocutaneous syndromes [1], the other three being  Sturge- Weber syndrome More Details More Details (encephalofacial angiomatosis), tuberous sclerosis and von Hippel- Lindau disease (retinocerebellar angiomatosis).

Neurofibromatosis is now recognized to consist of distinct variants that differ from each other genetically, microscopically and clinically. Nearly 50% of the cases show a definite familial predilection, by an autosomal dominant transmission, while the other 50% of patients show a negative familial trait owing to chromosomal mutations.

Though nine types are described by Riccardi and Gorlin et al , the most common form of NF is type I or von Recklinghausen's disease which constitutes 80-95% of the cases.

NF-1 or von Reckhnghausens disease (after the German pathologist who first documented the classical features of NF-1) has the following classical features

> Cafandaulaitspots

> Multipleneurofibromas

> Hamartomasoftheiris (lischnodules)

> Axillary/Inguinal freckling (Crowe's sign). Neurofibromatosis type It (NF-II) is a much more uncommon manifestation that probably results from a structural defect in chromosome 22, as opposed to NF-I, which is related to chromosome 17.

NF-II presents with predominant involvement of central nervous system with features such as

> Bilateral acoustic neuromas > Meningioma

> Ependyomas

> Sub capsular opacities of eye > Cafandaulaitspots

In addition to the above two forms, seven other variants of the disease are also described with varying features and involvement of other syndromes such as Noonan syndrome intype 9.

Apart from the above mentioned features, the systemic involvement of the disease also include the various organ systems of the body (cardiovascular, gastrointestinal, endocrinal, genitourinary and central nervous system)[6]. The oral manifestations of the disease include enlarged fungiform papilla being the most common, followed by single or multiple neurofibroma oftongue (macroglossia), floor of mouth, buccal mucosa, edentulous ridge, gingiva and palate. Intraosseous lesions can be seen with bone changes such as hypo and hyperplasia of basal bones involving mandible, maxilla, zygoma and TMJ [1].

The bone changes normally seen in the mandible are > Enlarged mandibularforamen

> Enlarged mental foramen > Osseous dysplasia

> Hyperostosis

> Branched mandibularcanal

As per the literature, around 40 cases have been reported as solitary intraosseous neurofibroma. An accurate incidence of solitary intraosseous neurofibroma is difficult to ascertain because of confusion with nemilemomma and neurofibromatosis [2]. Majority ofthe intraosseous forms reported are in the posterior mandible and a few in the maxilla, with the female to male ratio being 2:1[2]. A higher incidence of intraosseous nemilemomas in the mandible than in the maxilla has been reported. This predisposition of intraosseous neurogenic tumors to occur in the mandible may be attributable to the long thick bundles of the inferior alveolarnerve.

Neurofibroma is basically heterogeneous collection of axons, fibroblasts, Schwann cells and other cells of varying proportions, which is a feature of both NF-1 and NF-II.

Diffuse proliferation of neural tissue in the dermis can result in the formation ofthese nodular lesions, which may be broad sessile pachydermatocele or pedrmculated fibromamolluscum.

The differences in the neurofibromas presented by NF-I and NF-II are attributed to different growth factors acting to accelerate the proliferation of the corresponding cells, increasing that component in the presenting neurofibroma For example, nerve growth factor, accelerating the proliferation of nerve cell component in NF-I and glial growth factor, accelerating the proliferation of glial cells (Schwann cells) in acoustic neuromas of NF-1I.

Neurofibroma can be divided into classic, cellular and myxoidtypes histopathologically The classic form of the disease characteristically shows interlacing bundles of elongated spindle shaped Schwann cells with wavy dark stained nuclei. Thin fibers of collagen with mucoid material separating them from cells. Mast cells, lymphocytes and rarely xanthoma cells are also seen.

The cellular type presents with highly cellular areas with cells arranged in whorls, fascicles or storiform pattern in uniform collagen matrix devoid of mucosubstance, resembling Antoni A areas of neurilemrnoma. Some times small neurites may be seen.

The myxoidtype isvery rare. Itusually presents ashighly myxoid. Hypocellularity is a feature of this form. Pools of acid mucopolysaccharides are present.

Complete surgical excision is often possible for solitary intraosseous neurofibroma, however multiple lesions or plexiform neurofibroma that are entwined with normal tissues presents problems to the surgeon because of difficulty in complete eradication and prevention of neurological deficits and hemorrhage as a complication is often encountered [7],[8].

Adekeye et al advocated the inj ection of boiling water into the lesion to minimize the bleeding. They also mention that intraosseous neurofibromas often require radical surgery including hemimandibulectomy and enbloc resection of the mandible [9].

White et al [8] proposed that early conservative treatment is indicated for plexiform neurofibroma of head and neck that produces cosmetic or functional problems.

On the other hand, complete surgical removal should not be attempted if the neurofibromas do not cause obvious impairment of function and if the patients condition would not be improved by surgery [9].

Surgical treatment of acoustic neurofibroma (in type II) is risky because of the possibility of damage to the VII cranial nerve. Martuza and Eldridge indicated that no treatment is warranted on patients with stable audiological tests and minimal symptoms [10].

Complete excision can be performed if the tumor is small and deteriorating or if the lesion is growing and if the patient has acceptable hearing on the other side. Radiation to NF type I lesions are not indicated. Sterotactic radiation therapy can reduce the size of the acoustic neuromas but does not result in hearing improvement [11].

Recent research suggests that rapamycin and pirfendone has been employed with favorable short-term response [12] but this is still under research.

The propensity of neurofibromas to progress to neurofibromatosis or the primary disease undergoing malignant transformation (6 to29%) has been reported in the past [4],[5]. This progressive nature of the disease emphasizes the importance of close monitoring of the patients presenting initially only with neurofibromas.[15]

   References Top

1.Shapiro SD, Abramovitch K, Vandis ML, Skocylas LL Langlias RP, Jorgenson RJ, Young RS, Riccardi VM: Neurofibromatosis oral and radiographic manifestation oral Surg Oral Med Oral Pathol, 66: 391-396,1988.  Back to cited text no. 1    
2.Mori H, Kakuta S, Yamagichi A, Nagumo M: Incidence of solitary intraosseous neurofibroma of maxilla, J Oral Maxillofac Surg: 638-642,1993.   Back to cited text no. 2    
3.Neville BW, Datum DO, Allen CM, et al : Oral and maxillofacial pathology, Philadelphia, PA, Saunders, 1995.  Back to cited text no. 3    
4.Ambrosia JDA, Langlias RP, Young RS: Jaw and skull changes in neurofibromatosis oral Surg Oral Med Oral Pathol, 391-396,1988.  Back to cited text no. 4    
5.Hustin J, Delaire Y, Loveraval and Sambreville: Unusual Intramandibular neural tumors oral Sorg Oral Med Oral Pathol, 71:593-596,1991.  Back to cited text no. 5    
6.Geist JR, Gardner DL, Stefenac SJ: Oral manifestation of neurofibromatosis type-1 and II oral Sorg Oral Med Oral Pathol, 73:376-382,1992.  Back to cited text no. 6    
7.Apostolidis C Anterriotis D and Rapadis D, Angelos: Solitary intra osseous neurofibroma of the inferior alveolar nerve a case report, J Oral Maxillofac Surg, 59:232-235, 2001.  Back to cited text no. 7    
8.White AK, Smith RJH, Brooke AT, Bigler CR, Shatter PR: Head and neck manifestation of neurofibromatosis, Laryngoscope, 96:732-737, 1986.  Back to cited text no. 8    
9.Adekye EO, Abisole A: Neurofibromatosis of the head and neck clinical presentation and treatment, J Oral Maxillofac Sorg,12: 78-84,1984.  Back to cited text no. 9    
10.MartuzaRL, Eldridge R: Neurofibromatosis 11, New Eng J Med, 318:684-688,1988.  Back to cited text no. 10    
11.Constantiono PD, Freidman CD, Pelzer HL Neurofibromatosis type It of the head and neck, Arch Otolaryngol Head Neck Surg,113: 380-383,1989.  Back to cited text no. 11    
12.JarshdoferR.BenjammR. Chemotherapy of bilateral head and neck sung, Otolaryngol Head Neck Sung, 98: 273-82,1988.  Back to cited text no. 12    
13.Ardent DM, Schaberg SL Meadows JT: Neuro fibromatosis of the head and neck clinical presentation and treatment, J Oral Maxillofac Surg, 12:78-84,1987.  Back to cited text no. 13    
14.Damjanov 1, Linder J: Anderson's pathology, (10 fsub ed.), Mosby-year book,1996.  Back to cited text no. 14    
15.Neville BW, Hann J, Narang R, Garen DP, Charleston: Oral nemofibrosarcoma associated with neurofibromatosis type I oral Sorg Oral Med Oral Pathol, 66:391-396,1988.  Back to cited text no. 15    

Correspondence Address:
N Vivek
Department of Oral and Maxillofacial Surgery, Meenakshi Ammal Dental College and Hospital, Chennai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.29874

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]

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