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Year : 2018  |  Volume : 14  |  Issue : 3  |  Page : 712-715

Intraosseous neurofibroma in a 13-year-old male patient: A case report with review of literature


Department of Oral and Maxillofacial Pathology and Microbiology, Dr. D. Y. Patil School of Dentistry, Navi Mumbai, Maharashtra, India

Date of Web Publication12-Jun-2018

Correspondence Address:
Dr. Sandhya Tamgadge
Department of Oral and Maxillofacial Pathology and Microbiology, D. Y. Patil School of Dentistry Nerul, Navi Mumbai, Maharashtra - 400 706
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.176173

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 > Abstract 


Neurofibroma is a benign tumor of nerve tissue origin, derived from the cells that constitute the nerve sheath. It is commonly found in soft tissues, but the intraosseous occurrence of solitary neurofibroma in the head and neck is comparatively rare, with the most common site being mandible. This article presents a case report of neurofibroma on the posterior right side of the mandible of a 13-year-old boy who is the youngest patient in the series along with a review of literature.

Keywords: Intraosseous neurofibroma, mandible, S-100, Schwann cells, younger age


How to cite this article:
Iqbal A, Tamgadge S, Tamgadge A, Chande M. Intraosseous neurofibroma in a 13-year-old male patient: A case report with review of literature. J Can Res Ther 2018;14:712-5

How to cite this URL:
Iqbal A, Tamgadge S, Tamgadge A, Chande M. Intraosseous neurofibroma in a 13-year-old male patient: A case report with review of literature. J Can Res Ther [serial online] 2018 [cited 2022 Aug 10];14:712-5. Available from: https://www.cancerjournal.net/text.asp?2018/14/3/712/176173




 > Introduction Top


Neurofibroma is a benign nerve sheath tumor of the peripheral nervous system. A solitary neurofibroma is a single lesion that occurs in an individual who does not have hereditary neurofibromatosis.[1] Most solitary neurofibromas of the oral cavity appear in the soft tissues. The intraosseous occurrence of neurofibroma in the head and neck is a rare entity, most common site being the mandible. The average age is 27.5 years, ranging between 15 and 45 years old. Bruce in 1954 gave the first description of solitary neurofibroma of the oral cavity. Since then only a few (<50) cases have been documented in the literature.[2],[3]


 > Case Report Top


A 13-year-old boy presented with a swelling in the posterior right region of the mandible. The patient first noticed the swelling 3 months ago which was asymptomatic and small in size and was increased gradually. He first visited a local hospital where he was referred to a local dentist for the same. Incisional biopsy was performed 1 month before the surgery, and it was reported as neurofibroma.

On extra oral examination, a diffuse swelling of 2.0 cm × 3.0 cm × 2.0 cm in dimension was observed. The extension of the swelling was from body of the mandible to posterior border of the ramus of the mandible anterosuperiorly and superoinferiorly from 1 to 2 cm below the ala-tragus line to the inferior border of the mandible. Overlying skin was normal [Figure 1].
Figure 1: Extraoral view - Shows diffuse swelling of 2.0 cm × 3.0 cm × 2.0 cm in dimension extending from body of the mandible to posterior border of the ramus anterosuperiorly and superoinferiorly from 1 to 2 cm below the ala-tragus line to inferior border of the mandible

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On palpation, it was hard nontender and fixed to the underlying tissue. Intraoral examination showed no significant swelling with no occlusal derangement [Figure 2].
Figure 2: Intraoral view shows no significant swelling and occlusal derangement

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On radiographic examination orthopantogram showed ill-defined radiolucency with respect to 46 and 47.

The three-dimensional reconstruction image shows the involvement of soft tissue with respect to 45, 46, and 47 regions [Figure 3]a and [Figure 3]b.
Figure 3: Orthopantomogram and three-dimensional reconstruction image. (a) orthopantomogram showed ill-defined radiolucency with respect to 46 and 47, and dense radio-opacity with respect to 46. (b) Three-dimensional reconstruction image shows enlargement of lesion

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Axial section showed a large corticated homogenous expansion on buccal side, perforation of buccal cortical plate and thinning of lingual cortical plate. The excisional biopsy was performed [Figure 4].
Figure 4: Axial section - Axial section showed a large corticated homogenous expansion on buccal side, perforation of buccal cortical plate and thinning of lingual cortical plate

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Microscopic examination of hematoxylin and eosin stained section showed lesional tissue consisting of connective tissue stroma composed of proliferating interlacing bundles of spindle-shaped cells that exhibit wavy nuclei. These cells were associated with delicate collagen bundle and variable amounts of the myxoid matrix [Figure 5]a and [Figure 5]b.
Figure 5: (a) Hematoxylin and eosin stained section showed connective tissue stroma composed of proliferating interlacing bundles of spindle shaped cells with wavy nuclei. These cells were associated with delicate collagen bundle and variable amounts of the myxoid matrix. (b) Immunohistochemistry was strongly positive for S-100 protein

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The patient revisited the hospital after 3 months. The prognosis was fair and there was paresthesia on the operated site, with no sign of recurrence. Some amount of bone formation was evident [Figure 6].
Figure 6: Follow-up radiograph-prognosis was fair after 3 months follow-up. New bone formation was evident with no sign of recurrence

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 > Discussion Top


Solitary neurofibroma is a rare benign nonodontogenic tumor, especially in the oral cavity. Clinically, oral neurofibromas usually appear as a Pedunculated or sessile nodule, with slow growth. They are usually painless, but pain or paresthesia may occur due to nervous compression. Although neurofibromas are often found in the soft tissue of head and neck region, central variant such as in the jaw bones of the mandible or maxilla are rare. In the oral cavity, the most frequent location is the tongue, although they may occur at any site, especially on the palate, cheek mucosa, and floor of the mouth. Intraosseous location of the mandible has been described as seen in our case. Intraosseous neurofibromas are reportedly rare because the bones do not contain myelinated nerves or nerve sheaths within their medullary space.

Neurofibroma occurring in the jaw bones characteristically presents as a single lesion, and rarely presents as multiple lesions. As per literature, only few cases of intraosseous neurofibroma have been published.

Ellis et al. have published an excellent study covering a total 35 intraosseous benign nerve sheath tumors occurring in jaws, 23 of these cases were neurofibroma and 12 neurilemmomas.[4] Das Gupta et al., reported 303 cases of benign nerve sheath tumors, and about 45% involved the head and neck region, and approximately 9% occurred in the oral cavity.[5] Polak et al. reported a case of solitary neurofibroma of the mandible. Polak et al. analysis of 66 cases of neurofibroma revealed the following distribution concerning the site of occurrence of neurofibroma in the head and neck area: Tongue, 12; palate, 12; mandibular ridge/vestibule, 15; maxillary ridge/vestibule, 9; buccal mucosa, 10; lip, 4; mandibular intrabony, 2; gingiva, 1; and floor of the mouth, 1.[6] In this series, only 29 cases of solitary neurofibroma of the mandible was found. When solitary neurofibroma occurred in the mandible, there was definite female predilection (2:1) and was most frequently localized in the posterior section of the mandible.[7] However, this case occurred in male patient. The average age of occurrence is 27.5 years ranging between 14 and 45 years old.[8] but case report of a 13-year-old boy which makes this case unique. Only few cases have been reported so far of intraosseous neurofibroma [Table 1].
Table 1: Review of cases of intraosseous neurofibroma of the jaws

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Solitary oral neurofibromas are usually treated by surgical excision, depending on the extent and the site. Surgical removal may result in recurrence; multiple recurrences have been associated with malignant transformation.[16]


 > Conclusion Top


Neurofibroma is a benign nerve sheath tumor in the peripheral nervous system. Most solitary neurofibroma in the oral cavity appears in the soft tissues. The intraosseous occurrence of solitary neurofibroma in the head and neck is comparatively rare, with the most common site being the mandible. Complete surgical removal is the treatment for solitary neurofibroma and recurrence is rare.[7] But because of the previous reports of malignant transformation of these lesions, long-term follow-up of patients should be considered. In conclusion, it is important for dentists to consider the intraosseous neurofibroma in the differential diagnosis of the jaw radiolucencies.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Sharma P, Narwal A, Rana AS, Kumar S. Intraosseous neurofibroma of maxilla in a child. J Indian Soc Pedod Prev Dent 2009;27:62-4.  Back to cited text no. 1
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2.
Neville DW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3rd ed. Philadelphia: W.B. Saunders; 2009. p. 528-9.  Back to cited text no. 2
    
3.
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. 3
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4.
Ellis GL, Abrams AM, Melrose RJ. Intraosseous benign neural sheath neoplasms of the jaws. Report of seven new cases and review of the literature. Oral Surg Oral Med Oral Pathol 1977;44:731-43.  Back to cited text no. 4
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5.
Das Gupta TK, Brasfield RD, Strong EW, Hajdu SI. Benign solitary Schwannomas (neurilemomas). Cancer 1969;24:355-66.  Back to cited text no. 5
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6.
Polak M, Polak G, Brocheriou C, Vigneul J. Solitary neurofibroma of the mandible: Case report and review of the literature. J Oral Maxillofac Surg 1989;47:65-8.  Back to cited text no. 6
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Vivek N, Manikandhan R, James PC, Rajeev R. Solitary intraosseous neurofibroma of mandible. Indian J Dent Res 2006;17:135-8.  Back to cited text no. 7
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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. 8
    
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Larsson A, Praetorius F, Hjörting-Hansen E. Intraosseous neurofibroma of the jaws. Int J Oral Surg 1978;7:494-9.  Back to cited text no. 9
    
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Mori H, Kakuta S, Yamaguchi A, Nagumo M. Solitary intraosseous neurofibroma of the maxilla: Report of a case. J Oral Maxillofac Surg 1993;51:688-90.  Back to cited text no. 10
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Poupard RJ, Mintz S. Solitary intrabony neurofibroma of the maxilla. J Oral Maxillofac Surg 1997;55:768-72.  Back to cited text no. 11
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Apostolidis C, Anterriotis D, Rapidis AD, Angelopoulos AP. Solitary intraosseous neurofibroma of the inferior alveolar nerve: Report of a case. J Oral Maxillofac Surg 2001;59:232-5.  Back to cited text no. 12
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Skouteris CA, Sotereanos GC. Solitary neurofibroma of the maxilla: Report of a case. J Oral Maxillofac Surg 1988;46:701-5.  Back to cited text no. 13
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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 2009;5:24.  Back to cited text no. 14
    
15.
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. 15
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16.
Rajendran R. Benign and malignant tumors of oral cavity. In: Rajendran R, Sivapathasundaram B, editors. Shafer's Textbook of Oral Pathology. 6th ed. Philadelphia: Elsevier; 2009. p. 198-200.  Back to cited text no. 16
    


    Figures

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

  [Table 1]


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