Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 406

 Table of Contents  
Year : 2020  |  Volume : 9  |  Issue : 1  |  Page : 63-66

Case report: Primary intraosseous neurofibroma of maxilla

Department of Oral Pathology and Microbiology, G. Pulla Reddy Dental College and Hospital, GPR Nagar, Kurnool, Andhra Pradesh, India

Date of Submission18-Sep-2018
Date of Acceptance17-Jan-2020
Date of Web Publication14-May-2020

Correspondence Address:
Dr. C N V Akhila
G. Pulla Reddy Dental College And Hospital, GPR Nagar, Kurnool, Andhra Pradesh
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and Permissions

The present article is a case report of solitary neurofibroma of oral cavity in a 50-year-old male patient. Patient reported with chief complaint of swelling in relation to upper front teeth region since 2 months. Based on clinical, radiographical, and histopathological findings the diagnosis of neurofibroma was made. Neurofibroma involving oral cavity as primary lesion is very rare in occurrence. It mainly involves tongue, palate, and buccal mucosa. Surgical excision is performed in the present case.

Keywords: Intraosseous neurofibroma, soft tissue tumor of palate, solitary neurofibroma

How to cite this article:
Reddy Y R, Rajinikanth M, Radhika B, Akhila C N. Case report: Primary intraosseous neurofibroma of maxilla. J NTR Univ Health Sci 2020;9:63-6

How to cite this URL:
Reddy Y R, Rajinikanth M, Radhika B, Akhila C N. Case report: Primary intraosseous neurofibroma of maxilla. J NTR Univ Health Sci [serial online] 2020 [cited 2020 Sep 26];9:63-6. Available from: http://www.jdrntruhs.org/text.asp?2020/9/1/63/284317

  Introduction Top

Neurofibroma is a benign tumor arising from peripheral nerve sheath. The symptoms of which can range from localized swelling to multiple cutaneous swellings affecting skin. Majority of neurofibromas occur as autosomal dominant type of inheritance with strong genetic predisposition. This article presents a case report of a solitary intraoral neurofibroma with no known family history and absence of other cutaneous manifestations.

  Case Report Top

A 50-year-old male patient reported to the department of oral medicine and radiology, G. Pulla Reddy Dental College, with chief complaint of pain and swelling in relation to upper front teeth region since 2 months. Extraorally mild diffuse ovoid swelling was present at the upper lip region with obliteration of normal contour of philtrum. No history of trauma was present. Lymph nodes were not palpable. On intraoral examination a diffuse swelling was present in relation to 11 and 21 in the mid line [Figure 1]. The surface of the lesion was smooth, normal in color with no signs of inflammation and ulceration. The lesion extended from labial mucosa to palatal surface with swelling occupying palatal rugae region, with obliteration of labial vestibule and frenum at the involved site. The swelling measured 3 × 2 cm in size labially [Figure 2]. On palpation the swelling was sessile, firm in consistency, and was fixed to underlying structures. Hard tissue examination revealed grade 1 mobility in relation to 12, 11, 21, and 22 and teeth involved were vital. Patient was known diabetic and is under medication. No relevant medical and dental history was reported. No other systemic manifestations were evident.
Figure 1: Intraoral view of the lesion

Click here to view
Figure 2: Radiolucent lesion measuring 34 × 34 × 30 mm involving anterior maxilla

Click here to view

OPG revealed radiolucency in relation to 11, 12, 21, and 22 with irregular borders. Resorption of roots of involved teeth is evident. Teeth with respect to 11 and 21 showed distal migration. CT was performed for the present case which revealed a well-defined round to oval expansile lytic lesion (measuring 34 × 34 × 30 mm) with partially calcified/corticated peripheral wall seen in the alveolar process of the anterior maxilla and anterior hard palate in the midline causing anterior contour bulge. Based on clinical and radiographic features a provisional diagnosis of leiomyoma, schwannoma, and hemangioma were considered.

Fine needle aspiration revealed negative aspiration. An incisional biopsy was done in the present case. The specimen was whitish grey in color with irregular borders and firm in consistency. The tissue was processed and H and E stained. Microscopic examination of the H and E stained tissue under 10 × revealed fibrocellular connective tissue with prominent nerve bundles. The nerve fibers were with wavy nuclei and connective tissue is densely arranged collagen fibers with inflammatory cell infiltrate predominantly lymphocytes. Immunohistochemistry findings were positive for S-100 protein and CD34.

Complete excision was performed in the present case. Excision was carried out under general anesthesia. Labial flap was elevated and a vestibular incision was given as shown in the figure [Figure 3]. Vertical and horizontal bony cuts were given to mobilize the tumor segment. Complete premaxillary segment was removed canine to canine [Figure 4]. After excision cauterization was performed to achieve hemostasis [Figure 5]. Nasolabial flap was sutured to palatal and labial mucosa. The intraoral soft tissue defect was reconstructed with nasolabial flap. Postoperative ryles tube was advised for 2 weeks [Figure 6]. After 1 month we planned for obturator.
Figure 3: Operative photograph with elevated labial flap

Click here to view
Figure 4: Completely excised specimen

Click here to view
Figure 5: Hemostasis was achieved through cauterization

Click here to view
Figure 6: Patient with ryles tube

Click here to view

  Discussion Top

Neurofibroma is a benign tumor arising from nerve tissue origin, derived from the cells which constitute the nerve sheath. Neurofibroma can arise either as a solitary lesion or as a component of neurofibromatosis type I (NF1, also called as von Recklinghausen's disease of skin).[1] Neurofibromatosis type I is a neuroectodermal tumors arising within multiple organs and is inherited as an autosomal-dominant inheritance. It is the most common type and accounts for about 90% of the cases, and is characterized by the occurrence of neurofibromas along the peripheral nerves and multiple cafe-au-lait spots.[2],[3] In 1988, National Institute of Health consensus Development Conference proposed a diagnostic criterion for neurobromatosis type 1, if a patient has two or more of the following findings[4]:

  1. Six or more cafe au lait macules
  2. Two or more neurofibromas of any type or one plexiform neurobroma
  3. Freckling in the axillary or inguinal regions
  4. Optic glioma
  5. Lisch nodule
  6. Distinctive osseous lesion such as sphenoid dysplasia
  7. Family history offirst-degree relative with neurobromatosis.

In the present case, patient showed a palatal neurofibroma and the other features were not evident. Shklar and Meyer classified neurofibroma as solitary and multiple. Multiple neurofibromas are associated with Neurofibromatosis type 1 (NF1), whereas solitary neurofibromas are not associated with NF1 and it occurs very rare in the oral cavity. For solitary neurofibroma, the exact cause is unknown. As many as 50% of cases are reported to be caused due to the spontaneous mutations.[5] The present case showed solitary neurofibroma with negative family history.

In the late 19th century, two subsets have been identified. One is associated with NF1, caused by a mutation of an NF1gene located on chromosomal segment on the long arm of chromosome 17 (17q11.2), which encodes a protein known as neurofibromin, which plays a role in neural cell signaling. The other is associated with Neurofibromatosis type 2 (NF2), caused due to the gene mutations of the tumor suppressor genes coding for schwannomin on chromosome 22q12.1.[6],[7]

World Health Organization (WHO) classified neurofibromas into two categories: dermal and plexiform. Dermal neurofibromas are associated with a single peripheral nerve, while plexiform neurofibromas are associated with multiple nerve bundles.[8]

Solitary neurofibromas appear as soft, painless, circumscribed, slow growing lesions that vary in size from small nodules to larger masses. The most frequent location for neurofibromas is the skin. Most common intraoral sites are the tongue and buccal mucosa followed by palate, gingiva, alveolar ridge, and vestibule.[9] The oral lesions appear as discrete, nonulcerated nodules, which tend to be of the same color as the normal mucosa. Due to the diffuse involvement of the tongue, macroglossia is evident. Few cases have been reported with involvement of submandibular gland, tongue, and lower lip.

In the present case, the intraoral examination revealed a diffuse swelling in relation to 11 and 21 in the midline. The surface of the lesion was smooth, normal in color with no signs of ulceration. The lesion extended from labial mucosa to palatal surface with swelling occupying palatal rugae region.

In the present case, the histopathology revealed the presence of bundles of nerve fibers with wavy nuclei and densely arranged collagen fibers with inflammatory cell infiltrate predominantly lymphocytes. Immunohistochemistry was done. Immunohistochemical findings were multiple, well-demarcated fascicles of spindle-shaped nerve cells, most of which are positive for S-100 protein. It showed moderate positive immunoreactivity to S-100 protein and CD34. CD34 is a useful stain for the differential diagnosis, since neurofibromas contain additional cellular components (endoneurial fibroblasts, perineurial-like cells, etc.) that are responsible for this CD34 reactivity.

Solitary oral neurofibromas are treated by surgical excision, depending on the site and extent. Recurrence may occur due to the surgical excision, and multiple recurrences have been associated with malignant transformation.

  Conclusion Top

Neurofibromas may occasionally manifest as solitary swellings in intraoral region, as primary lesion. Appropriate diagnostic and surgical approach should be carried out in a case of neurofibroma, as done in the present case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Njoumi N, Elabsi M, Attolou G, Elouazzani H, Elalami FH, Chkoff MR. Solitary preperitoneal neurofibroma: A case report. BMC Res Notes 2015;8:115.  Back to cited text no. 1
Borges AH, Correia Rde M, Borba AM, Guedes OA, Estrela CR, Bandeca MC. Unusual solitary neurofibroma on the lower lip of a child. Contemp Clin Dent 2013;4:512-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
Tao Q, Wang Y, Zheng C. Neurofibroma in the left mandible: A case report. Kaohsiung J Med Sci 2010;26:217-2.  Back to cited text no. 3
Bharath TS, Krishna YR, Nalabolu GR, Pasupuleti S, Surapaneni S, Ganta SB. Neurofibroma of the palate. Case Rep Dent 2014;2014:898505. doi:10.1155/2014/898505.  Back to cited text no. 4
Mahalle A, Gs Reddy M, Mohit Kheur S, Bagul N, Ingle Y. Solitary non syndromic oral plexiform neurofibroma: A case report and review of literature. J Dent (Shiraz) 2016;17:293-6.  Back to cited text no. 5
Ohno J, Iwahashi T, Ozasa R, Okamura K, Taniguchi K. Solitary neurofibroma of the gingiva with prominent differentiation of Meissner bodies: A case report. Diag Pathol 2010;5:61-4.  Back to cited text no. 6
Narang BR, Palaskar SJ, Bartake AR, Pawar RB, Rongte S. Intraosseous neurofibroma of the mandible: A case report and review of literature. J Clin Diag Res 2017;11:6-8.  Back to cited text no. 7
Suramya S, Shashikumar P, Shreeshyla HS, Kumar GS. Solitary plexiform neurofibroma of the gingiva: Unique presentation in the oral cavity. J Clin Diagn Res 2013;7:2090-2.  Back to cited text no. 8
Lykke E, Noergaard T, Rasmussen ER. Lingual neurofibroma causing dysaesthesia of the tongue. BMJ Case Rep 2013;22:461-4.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


Similar in PUBMED
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded43    
    Comments [Add]    

Recommend this journal