CASE REPORT

Solitary Neurofibroma of the Tongue

KAJLA P.1, LATA J.2 , BANSAL P.3

ABSTRACT-

Neurofibroma is an uncommon benign tumor of the oral cavity derived from the cells that

constitute the nerve sheath. The oral cavity involvement by a solitary and peripheral

plexiform neurofibroma in patients with no other signs of neurofibromatosis is uncommon.

Amongst the histological variants, plexiform types are considered exclusive. These are poorly

circumscribed, locally invasive and may exhibit sarcomatous potential. Plexiform

neurofibromas are key features of Neurofibromatosis – 1 and their solitary intra–oral

presentation is uncommon.

The following case report describes isolated solitary plexiform neurofibroma of tongue in a

middle aged female patient.

Key words: Neurofibroma, Plexiform, Tongue

INTRODUCTION

Neurofibromas are benign tumors arising from peripheral nerve sheaths that affect the head

and neck region only rarely,1 and consist of Schwann cells, perineural cells, and a variable

amount of mature collagen.2 They are classified as solitary when not associated with any

syndrome or multiple when associated with autosomal dominant neurofibromatosis syndrome

or type III multiple neoplastic syndrome;3 They can also be classified as myxomatous (solid,

central, diffuse) or plexiform (peripheral). 4

Localized or solitary neurofibroma is the most frequent manifestation and develops along a

peripheral nerve as a focal mass with well defined margins but is never encapsulated.

Localized or solitary neurofibroma is rare in infancy and typically appears in late childhood

or during teenage years. Solitary plexiform neurofibromas are exceedingly rare in oral cavity.

Most common sites are buccal region, palate,tongue and lips5-10

Past literature reports that the frequency of solitary neurifibromas in the oral cavity remains

6.5% especially in lesions not associated with neurofibromatosis type -1 11. Also, the presence

of a plexiform neurofibroma in the oral cavity is rare.3

Morphologically, the tumor is composed of small axons among the proliferating Schwann

and perineural cells,supported by collagen matrix.8 The treatment of choice is complete

excision. The nerve from which the tumours originates may have to be resected in cases

where the lesions cannot be dissected.6 Recurrence is rare.6,9

CASE REPORT

A 35 years old female patient (Fig.1) reported to the outpatient department with a chief

complaint of increased mass at the right lateral border of tongue for the past 3 yrs. (Fig.2)

Since then, there had been gradual increase in size of the growth to its present size. The

lesion was asymptomatic as there was no history of associated pain, bleeding or any

impairment of function

On physical examination the patient was otherwise healthy. Medical history of the patient

was insignificant. Also, no pertinent family history of the similar growths was reported.

On inspection solitary swelling was seen extending 1cm behind from tip of tongue in

horizontal direction to right lateral posterior surface of tongue measuring about 3.5 cm

(Fig.3). In vertical direction it was extending from dorsal surface to the ventral surface of

tongue measuring about 2.2 cm (Fig.4). Swelling was sessile and irregular in shape with well

defined edges and smooth surface. Overlying mucosa was normal in color.

On palpation, the swelling was normal in temperature, non tender, firm in consistency, non

fluctuant and non compressible with no evident discharge. It had gradually increased to

present size within a year of approx 2.5 yr.

No anaesthesia or paresthesia of the lingual nerve distribution was found.No deviation of

tongue on protrusion.

Fig.1. Preoperative front view Fig.2. Preoperative view showing tongue growth

Fig.3. Preoperative view horizontal extent Fig.4. Preoperative view vertical extent Considering the clinical presentation and localization of the lesion, we included, reactive

lesions, such as giant cell fibroma or focal fibrous hyperplasia, neurilemmoma, granular cell

tumor , neurofibroma in the differential diagnosis.

On the basis of the diagnostic possibilities and considering the probable benign nature of the

lesion, excisional biopsy was planned.

The growth was surgically excised under local anaesthesia using 2% lignocaine with 1:

200,000 Adrenaline along with healthy margins. Primary closure was done with 3-0 Vicryl

Suture. The patient was put under analgesics and antibiotics for three days. Post operative

healing was uneventful.

The excised mass (Fig.5) was washed with 0.9 % normal saline, stored in 10 % formalin and

sent to the department of Pathology for routine histopathological evaluation.

On histopathologic examination the H and E stained sections showed superficial stratified

squamous parakeratinized epithelium. Connective tissue showed presence of tumour mass

consisiting of cells arranged in fascicles. The cells were elongated and spindle shaped

consisiting of thin wavy nucleus and scanty cytoplasm. Presences of numerous nerve bundles

were noted. The surrounding stroma consisted of connective tissue fibrils, fibroblasts and few

blood vessels (Fig.6.) Based on these findings the definitive diagnosis of Plexiform

Neurofibroma was given.

During the follow up period of approx 1 year no signs of recurrence were noted.

Fig.5. Excised growth Fig.6. Histopathological view

DISCUSSION

Neurofibromatosis is a hereditary condition resulting in a widespread developmental defect

of the nerve sheath 12. Although, the disease became widely recognized as a pathological in

the late 19th century: it was only recently, that its two subsets have been defined 13. They are

associated with Neurofibromatosis type–1 and Neurofibromatosis type–2 9.The former is

more common and accounts for about 90% of the cases .14

Neurofibromas are benign tumours of nerve cell origin. They are mostly seen as a part of

neurofibromatosis–I and the presence as a solitary condition is uncommon. The World Health

Organization (WHO) has subdivided neurofibromas into 2 broad categories: dermal and

plexiform. Dermal neurofibromas arise from a single peripheral nerve, while plexiform

neurofibromas are associated with multiple nerve bundles. Other clinicopathologic subtypes

include localized neurofibroma (sporadic neurofibroma), diffuse neurofibroma, plexiform

neurofibroma, and epithelioid neurofibroma .15

Solitary neurofibroma is a benign, slowly growing, relatively circumscribed, but non

encapsulated tumor diagnosed by absence of other features of the associated systemic

disease. Oral lesions are associated with as much as 72% of the multiple form in patients

with neurofibromatosis. Intra-oral presentation is extremely rare in case of the solitary type of

neruofibromas.14

It is believed that the frequency of oral solitary neurofibromas not associated with

neurofibromatosis-1 is low.4 They are slow -growing, nodular, sessile, and mobile tumors,3

usually painless, although pain or numbness may occur due to nerve compression. Cherrick

and Eversole 2 observed a higher incidence in females. Chen and Miller reported that oral

neurofibromas affect individuals between 9 and 72 years of age.16

Although some lesions require imaging tests to determine possible extension, conventional

histological analysis is conclusive. Microscopically the tumor is composed of an irregular

pattern of proliferative spindle cells. The stroma is composed of collagen fibers and mucoid

masses. Small axons all over the tumoral tissue are demonstrated with silver staining.

Neurofibromas are immunopositive forthe S-100 protein in 85 to 100% of the cases,

indicating its neural origin. 17-19

Treatment of choice is surgical excision of the solitary lesions, trying to conserve the nerve

from which the tumor originates 3. Neurofibromas may exhibit sarcomatous alteration in 3%–

15% of cases; especially in multiple neurofibromatosis 3. Occasionally, the malignant

transformation of Plexiform Neurofibroma is reported. These have poor prognosis and are

designated as a malignant peripheral nerve sheath tumor 15.

The present case is unique as the presentation of lesion was sporadic and no associated family

history was reported. A thorough examination of the patient was performed for the various

manifestations of neurofibromatosis–1 (like cafe – au lait spots, lisch nodules, axillary

freckling etc). The disease was ruled out due to the absence of the same. The patient had

reported with no complications over the follow up period of 12 months and is kept under

observation.

References

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2. Cherrick HM, Eversole LR. Benign neural sheath neoplasm of the oral cavity. Report of thirty-seven cases. Oral Surg Oral Med Oral Pathol. 1971; 32:900-9.

3. Alatli C, Oner B, Unur M, Erseven G. Solitary plexiform neurofibroma of the oral cavity. A case report. Int J Oral Maxillofac Surg. 1996; 25:379-80.

4. Marocchio LS, Oliveira DT, Pereira MC, Soares CT, Fleury RN. Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years. Clin Oral Investig. 2007; 11:165-9.

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11. Marcia Sampaio Campos, et al. Clinicopathologic and immunohistochemical features of oral neurofibroma. Acta odontologica scandinavica. 2012; 70:577- 82.

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13. Ahn MS, et al. The early history of the neurofibromatosis. Evolution of the concept of neurofibromatosis type 2. Archives of Otolaryngology - Head and Neck Surgery. 1996 Nov;122(11):1240-9.

14. NP Zwane, et al. Solitary oral plexiform neurofibroma: review of literature and report of a case. Oral oncology. 2011: 47; 449-51.

15. Indraneel Bhattacharya. Oral neurofibroma. Medscape reference-Drugs, diseases and procedures: Update August 2011

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17. Fisher DA, Chu P, McCalmont T: Solitary plexiform neurofibroma is not pathognomonic of von Recklinghausen's neurofibromatosis: a report of a case. Int J Dermatol 1997, 36:439-442.

18. Johnson MD, Glick AD, Davis BW: Immunohistochemical evaluation of Leu-7, myelin basic-protein, S100-protein, glialfibrillary acidic-protein, and LN3 immunoreactivity in nerve sheath tumors and sarcomas. Arch Pathol Lab Med 1988,112:155-160.

19. Weiss SW, Langloss JM, Enzinger FM: Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. Lab Invest 1983, 49:299-308.

PARTICULARS OF CONTRIBUTORS:

1. Demonstrator, Dept of Oral and Maxillofacial Surgery, Punjab Govt Dental College, Amritsar.

2. Professor & Head, Dept of Oral and Maxillofacial Surgery, Punjab Govt Dental College, Amritsar.

3. Post graduate student, Dept of Oral and Maxillofacial Surgery, Punjab Govt Dental College, Amritsar.

NAME, ADDRESS, E-MAIL ID OF THE CORRESPONDING AUTHOR:

NAME- DR PRATEEK BANSAL

CURRENT POSITION- POST GRADUATE STUDENT, DEPT OF ORAL AND MAXILLOFACIAL SURGERY

AFFILIATION INSTITUTION- PUNJAB GOVERMENT DENTAL COLLEGE, AMRITSAR (PUNJAB)

ADDRESS- ROOM NO 3, DEPT OF ORAL AND MAXILLOFACIAL SURGERY, 4S CHAWK, PUNJAB GOVT DENTAL COLLEGE, AMRITSAR,PUNJAB(143001)

MOBILE NUMBER- 7355495950, 8146053897

EMAIL ADDRESS- drprateekbansal25@gmail.com