|Year : 2015 | Volume
| Issue : 1 | Page : 136-139
Neurofibroma of mandible: A case report
Siddharth Kumar Singh, Nandika Babele, Tushar Phulambrikar, Anjali Gupta
Department of Oral Medicine and Radiology, Sri Aurobindo College of Dentistry, Indore, Madhya Pradesh, India
|Date of Submission||23-Nov-2014|
|Date of Acceptance||14-Jul-2015|
|Date of Web Publication||12-Oct-2015|
Department of Oral Medicine and Radiology, Sri Aurobindo College of Dentistry, Bhawrasla, Indore - 452 001, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Neurofibroma is a benign neoplasm of non-odontogenic origin arising from peripheral nerves. It may occur as a solitary lesion or as a part of the generalized syndrome of neurofibromatosis or von Recklinghausen's disease of the skin. It occurs rarely in the head and neck region; intraorally, it affects the tongue, buccal mucosa, and vestibular area, with the posterior mandible being the most common intraosseous location. Clinically, oral neurofibromas usually appear as pedunculated or sessile nodules with slow growth and are usually asymptomatic. The diagnosis can be confirmed by histological examination. Treatment is surgical with excellent prognosis. For illustration, a rare case of a solitary neurofibroma in the mandible is presented.
Keywords: Benign, mandible, neurofibroma, tumor of neural origin
|How to cite this article:|
Singh SK, Babele N, Phulambrikar T, Gupta A. Neurofibroma of mandible: A case report. J Indian Acad Oral Med Radiol 2015;27:136-9
|How to cite this URL:|
Singh SK, Babele N, Phulambrikar T, Gupta A. Neurofibroma of mandible: A case report. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2020 Aug 11];27:136-9. Available from: http://www.jiaomr.in/text.asp?2015/27/1/136/167136
| Introduction|| |
Neurofibroma is the most common type of peripheral nerve neoplasm. It is a benign tumor which may present either as a solitary lesion or as part of a syndrome, i.e. neurofibromatosis or von Recklinghausen's disease of the skin.  The skin is the most frequent location for neurofibromas, with tongue and buccal mucosa being the most common intraoral sites. Rarely, the tumor arises centrally within the bone, producing a well-defined or ill-defined, unilocular/multilocular radiolucency, with mandible being a common site for such an occurrence.  The first description of solitary neurofibroma of the oral cavity was given by Bruce in 1954, and until now, only a few cases have been reported in the literature.  A rare case report of a solitary neurofibroma involving the mandible is hereby presented.
| Case Report|| |
A 10-year-old boy reported to the Department of Oral Medicine and Radiology with a chief complaint of swelling on the left side of his face since he was 3-4 years old. It was initially small and then gradually grew to its present size. He did not take any treatment for it since it was asymptomatic. He visited the department after the swelling became extensive. On examination, it was a large swelling on the left side of the face that extended lateromedially from the left ear lobule, crossing the midline and extending to the right corner of mouth and superoinferiorly from the infraorbital margin to 3 cm below the lower border of mandible approaching the neck region [Figure 1]. The skin overlying the swelling was stretched with pigmentation on the lateral aspect. Temperature of the skin was normal. On palpation, it was a soft cystic swelling, compressible, measuring about 14 × 10 cm in dimension, non-fluctuant, and non-tender. There was no neurovascular compromise in that particular area. Intraoral examination revealed a soft swelling obliterating the vestibule, involving the left alveolus with the displacement of the teeth lingually leading to malocclusion. There were several carious teeth in the oral cavity due to poor oral hygiene. The provisional diagnosis according to the history and clinical presentation was made as ameloblastoma.
Radiological investigations were done following the clinical examination. These included the ultrasonography of the soft tissue mass (local region on left neck) which showed erosion of the underlying mandible with periosteal elevation along with moderate vascularity within the soft tissue compartment. The lymph nodes of the upper jugular chain on the left side were also enlarged. Computed tomographic (CT) scan [Figure 2], [Figure 3], [Figure 4] and [Figure 5] revealed a very large soft tissue mass with corticated and sharply defined borders, measuring 10.6 × 10 × 8.2 cm in dimension, arising from the left side of the mandible, causing erosion of the underlying cortex with attenuation values in the range of 40-60 HU. No evidence of any calcification, floating tooth, or destroyed bony fragment was found within the lesion. Enlarged lymph nodes were seen along the left carotid sheath that measured between 0.5 and 2.5 cm in dimension. The right side of mandible and the maxillary bone appeared to be normal. MRI picture revealed well-defined smooth margin with altered signal intensity arising from the left side of mandible projecting to the left lateral side. The lesion exhibited isointense signal on T1-weighted images and mixed hypointense and slight hyperintense signal on T2-weighted images.
Based on the clinical and radiographic findings, fibrous dysplasia, ameloblastic fibroma, odontogenic myxoma, neurofibroma, neurilemmoma, schwannoma, and central hemagioma were proposed as differential diagnoses. Other investigations were then subsequently carried out. Hemogram was within normal limits except for an increased value of absolute eosinophil count (540 mm 3 ). The HBsAg antigen and HIV tests were negative. Fine needle aspiration cytology (FNAC) from the neck swelling was non-specific and showed only the presence of red blood cells. Incisional biopsy from the lesion revealed a submucous benign mesenchymal proliferation with no signs of malignancy. Thus, surgical excision of the lesion was planned under general anesthesia (GA) and the specimen was sent for histopathologic examination. The macroscopic picture of the excised lesion showed a well-encapsulated, firm, nodular mass measuring 13 × 9 × 6 cm and weighing 500 g. Cut section showed solid, grayish white surface with stromal appearance and vague lobulation. Microscopically it showed a plexiform pattern containing distorted masses of peripheral nervous tissue scattered in collagenous matrix. There were no features of cellular atypia. Neurofibroma was thus, given as the final diagnosis. The patient was kept on a periodic recall for follow-up.
| Discussion|| |
Neurofibroma is one of the most common nerve sheath tumors occurring in soft tissues. Ninety percent of the neurofibromas are associated with neurofibromatosis type I.  According to Regezi,  intraoral neurofibromas may be seen in 25% of the patients with neurofibromatosis, while Preston  reported its presence in 7% of patients. Thus, the presence of a solitary case requires physical examination and family history so as to exclude its connotation with neurofibromatosis. In this case, there were no clinical signs or family history suggestive of neurofibromatosis. The lesion was a solitary one. Solitary lesions are most common in young adults and they present as slow-growing, soft, painless lesions varying in size from small nodules to massive masses.  There is no sex predilection.  The lesions occurring in the oral cavity are mostly solitary, with tongue, buccal mucosa, and lips being most commonly involved.  Central involvement particularly in jaw bones is quite unusual because bones do not contain medullary nerves or nerve sheaths within their medullary spaces.  The central lesions present with varying clinical symptoms ranging from relatively small asymptomatic lesions to extensive lesions causing expansion of the underlying bone, with or without destruction, and in some cases leading to pain and anesthesia or paresthesia of the area involved. The diagnosis is made by histological examination which exhibits interlacing bundles of elongated cells with wavy nuclei intimately associated with wire-like strands of collagen composed of a varying number of cells, among which are recognized the Schwann cells, perineural cells, fibroblasts, and intermediate cells. Immunohistochemical examination is done to confirm the diagnosis, which stands positive for the S-100 protein in 85-100% of the cases, indicating its neural origin.  Surgical excision is the main treatment modality for treating neurofibromas, but complete excision of these benign tumors may result in greater morbidity due to the complicated anatomy. Other alternatives include radiotherapy for localized lesions, image-guided radiation therapy (IGRT),  and photodynamic therapy.  Recurrence of the lesions is rare, although some authors suggest higher rate of recurrence at head and neck location of solitary neurofibromas.  Thus, the patient should be kept on regular follow-ups for periodic evaluation to check for recurrence and also to rule out its malignant transformation.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]