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Year : 2020  |  Volume : 63  |  Issue : 2  |  Page : 276-278
Solitary intraosseous neurofibroma of the mandible: Report of an extremely rare histopathologic feature

1 Department of Oral and Maxillofacial Pathology, Dental School, Semnan University of Medical Sciences, Semnan, Iran
2 Australian Research Centre for Population Oral Health (ARCPOH), Adelaide Dental School, University of Adelaide, Adelaide, South Australia, Australia

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Date of Web Publication18-Apr-2020


Neurofibroma (NF) is a benign tumor derived from the peripheral nerve sheath. Neurofibromas may present either as solitary lesions or as part of the generalized syndrome of neurofibromatosis or von Recklinghausen's disease of the skin. The intraosseous variant of NF is very rare. We report a case of a 32-year-old female who was diagnosed with a solitary intraosseous neurofibroma of the mandible. The present case is rare with respect to its unique histopathologic feature.

Keywords: Benign tumor, intraosseous, mandible, nerve sheath, solitary neurofibroma

How to cite this article:
Behrad S, Sohanian S, Ghanbarzadegan A. Solitary intraosseous neurofibroma of the mandible: Report of an extremely rare histopathologic feature. Indian J Pathol Microbiol 2020;63:276-8

How to cite this URL:
Behrad S, Sohanian S, Ghanbarzadegan A. Solitary intraosseous neurofibroma of the mandible: Report of an extremely rare histopathologic feature. Indian J Pathol Microbiol [serial online] 2020 [cited 2021 Nov 27];63:276-8. Available from: https://www.ijpmonline.org/text.asp?2020/63/2/276/282687

   Introduction Top

Neurofibroma is a benign, well-circumscribed soft tissue tumor of the peripheral nerve sheath phenotype with mixed cellular components which includes Schwann cells, perineural hybrid cells, and intraneural fibroblasts.[1],[2],[3],[4] Neurofibroma occurs as a single tumor or associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetically inherited disease.[1],[2],[3],[4],[5],[6] A solitary neurofibroma is a single lesion that occurs in a patient who does not have neurofibromatosis.[7] Neurofibromas are mostly observed on the skin and very few cases of neurofibromas have been reported in the oral cavity.[1],[2],[4] The tongue, buccal mucosa, lip, palate, gingival, and major salivary glands are the most common intraoral sites whereas intraosseous or central neurofibromas of the mandible are very rare.[1],[2],[4],[5] In 1954, Bruce gave the first description of solitary of the oral cavity. Since then, less than 50 cases have been documented in the literature.[8]

We report a case of an intraosseous neurofibroma of the mandible in a 32-year-old female patient who showed a unique histological feature of the tumor.

   Case Report Top

A 32-year-old female patient visited the department of oral medicine of Semnan dental school with a chief complaint of swelling in the lower left side of the face. The patient gave a history of a slow-growing swelling for 6 months with intermittent dull aching pain for the past 3 months. Intraorally there was diffuse swelling in the lingual cortex [Figure 1]. The patient had no medical history and systemic diseases.
Figure 1: (a) Clinical feature showed swelling in the lower left side of the face (b) Intraoral feature showed diffuse swelling in the lingual cortex

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On panoramic radiography and CT scan image, there was a well-defined unilocular radiolucency in the left side of the mandible measuring about 3 × 1.5 × 2.4 cm. Lingual cortical expansion and thinning of buccal and lingual cortexes were seen. There was no evidence of root resorption. The loss of lamina dura and PDL widening of the second molar was obvious. Cortical borders of the mandibular canal were lost [Figure 2].
Figure 2: (a) Panoramic radiography showed well-defined unilocular radiolucency in the left side of the mandible (b) CT images

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Incisional biopsy was done and the histopathological examination revealed a benign proliferation of spindle-shaped cells with wavy nuclei and densely packed collagen bundles within the fibromatous stroma. The diagnosis of neural tumors was given which was followed by excisional biopsy. A similar histopathological feature was seen in excisional biopsy tissue [Figure 3]. Accordingly, the diagnosis of neurofibroma was confirmed.
Figure 3: (a) Microscopic feature of the incisional biopsy showed benign proliferation of spindle-shaped cells with wavy nuclei and densely packed collagen bundles within the fibromatous stroma. (H and E × 100) (b) Microscopic feature of incisional biopsy (H and E × 400) (c) Microscopic feature of the excisional biopsy showed benign proliferation of spindle-shaped cells with wavy nuclei and densely packed collagen bundles within the fibromatous stroma (H and E × 100) (d) Microscopic feature of excisional biopsy (H and E × 400)

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Considering the location of the tumor and radiographical and histopathological features intraosseous neurofibroma was diagnosed.

   Discussion Top

Neurofibroma is a benign soft tissue tumor that originates from the nerve sheath cells.[1] NF is presented as a solitary tumor or as multiple lesions associated with neurofibromatosis type 1 (NF1).[2] In the oral cavity, neurofibroma occurs on the tongue, lip, palate, gingiva, salivary glands, and the jawbones.[2],[3],[4]

A literature search of intraosseous NFs of the jaws showed few reported cases. Che et al. noticed the number of NFs occurring in the posterior part of the mandible, which could be the reason for the passing of bundles of inferior alveolar nerve in the mandibular canal.[2],[9] Neurofibroma occurs at various ages between 14 and 45 years old. A male: female ratio of 1:2 has been observed.

Most of the intraosseous NFs are asymptomatic in the initial stages. As the tumor increases in its size, it starts compressing on the adjacent vital structures and begins to destroy the bone.[10] Later on, pain and numbness of the affected side of the lip occur. So far, few cases of symptomatic intraosseous neurofibroma have been reported.[9] Similar to the symptoms reported in the literature search, the present case also depicted the symptoms of swelling and bone loss.[2],[4],[5]

Radiologically, the tumor appears as a nonspecific, unilocular or multilocular, poorly defined or well-demarcated, radiolucency.[6] Alatli et al. reported an intraosseous neurofibroma with no abnormality in radiographic features.[11]

Histopathologically, NFs exhibit an irregular pattern with interlacing bundles of spindle-shaped cells with round or fusiform nuclei and eosinophilic cytoplasm within a loose matrix of delicate fibrillary collagen and a variable amount of myxoid matrix.[1],[2],[12] Ide et al.[13] also recognized that neurofibroma is composed of a complex proliferation of Schwann cells, perineural cell, endoneurial fibroblasts, and intermediate cells. The researcher's distinguished three types of neurofibromas (type I, II, and III) based on their reactivity to different markers and ultrastructural features. This subdivision is useful and represents the variable possibility for different markers. This case should be considered as an intraosseous neurofibroma of controversial diagnosis because it showed no histological features typical of NF. The histopathology feature of this case is remarkable because it showed a fibro collagenous stroma rather than myxoid matrix, and it was similar to some cases which had been reported as central NF.[12],[14],[15],[16],[17],[18],[19],[20],[21]

It is important to differentiate NFs from other spindle cell tumors such as Schwannoma. Although both have a neural origin, there are slight anatomical considerations associated with them. Neurofibroma attempt to encase the nerve fiber while Schwannomas will typically displace the root of nerve they are associated with.[7]

Neurofibromas are immunoreactive for S100 protein, indicating its neural origin.[1],[2] Mast cells are numerous and can be a helpful diagnostic feature.[1] Despite the above features and considering the histopathological architecture of the lesion, the anatomic area (periphery of the inferior alveolar nerve), and the biological behavior, we confirm the diagnosis of neurofibroma.

Complete surgical excision is often possible for solitary intraosseous neurofibroma. On the other hand, complete surgical removal should not be attempted if the neurofibromas do not cause obvious impairment of function and if the patient's condition would not be improved by surgery. Complete excision can be performed if the tumor is small and deteriorating or if the lesion is growing and if the patient has an acceptable hearing on the other side.[22],[23],[24]

   Conclusion Top

Neurofibromatosis is a relatively common disorder but intraosseous neurofibroma is a rarity. As a solitary lesion, it might present diagnostic difficulties. Hence, it is essential for oral diagnosticians to be aware of even the rare presentations of relatively common disorders which might also be the first indicator of the disease process.

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 b'e 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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Neville BW, Damm DD, Chi AC, Allen CM. Oral and Maxillofacial Pathology. Elsevier Health Sciences; 2015.  Back to cited text no. 1
Gujjar PK, Hallur JM, Patil ST, Dakshinamurthy SM, Chande M, Pereira T, et al. The solitary variant of mandibular intraosseous neurofibroma: Report of a rare entity. Case Rep Dent 2015;2015:520261.  Back to cited text no. 2
Deichler J, Martínez R, Niklander S, Seguel H, Marshall M, Esguep A. Solitary intraosseous neurofibroma of the mandible. Apropos of a case. Med Oral Patol Oral Cir Bucal 2011;16:e704-7.  Back to cited text no. 3
Narang BR, Palaskar SJ, Bartake AR, Pawar RB, Rongte S. Intraosseous neurofibroma of the mandible: A case report and review of literature. J Clin Diagn Res 2017;11:ZD06-8.  Back to cited text no. 4
Ueda M, Suzuki H, Kaneda T. Solitary intraosseous neurofibroma of the mandible: Report of a case. Nagoya J Med Sci 1993;55:97-101.  Back to cited text no. 5
Dalili Z, Adham G. Intraosseous neurofibroma and concurrent involvement of the mandible, maxilla and orbit: Report of a case. Iran J Radiol 2012;9:45-9.  Back to cited text no. 6
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Bruce KW. Solitary neurofibroma (neurilemmoma, schwannoma) of the oral cavity. Oral Surg Oral Med Oral Pathol 1954;7:1150-9.  Back to cited text no. 8
Che Z, Nam W, Park WS, Kim HJ, Cha IH, Kim HS, et al. Intraosseous nerve sheath tumors in the jaws. Yonsei Med J 2006;47:264-70.  Back to cited text no. 9
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Alatli C, Öner B, Ünür M, Erseven G. Solitary plexiform neurofibroma of the oral cavity: A case report. Int J Oral Maxillofac Surg 1996;25:379-80.  Back to cited text no. 11
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Ide F, Shimoyama T, Horie N, Kusama K. Comparative ultrastructural and immunohistochemical study of perineurioma and neurofibroma of the oral mucosa. Oral Oncol 2004;40:948-53.  Back to cited text no. 13
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Gutman D, Griffel B, Munk J, Shohat S. Solitary neurofibrcma of the mandible. Oral Surg Oral Med Oral Pathol 1964;17:1-9.  Back to cited text no. 19
Villa VG, Laico JE, Bañez LO. Central neurofibroma in the mandible associated with occasional spontaneous hemorrhage: Report of a case. Oral Surg Oral Med Oral Pathol 1962;15:836-42.  Back to cited text no. 20
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[PUBMED]  [Full text]  
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Correspondence Address:
Shabnam Sohanian
17 Shahrivar Blvd, Department of Oral and Maxillofacial Pathology, Dental School, Semnan University of Medical Sciences, Semnan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_28_19

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