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Neurofibroma the Benign Tumor of Oral Cavity: A Case Report

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AMRIT, 1(1), 2022, 61-63 ISSN

CASE REPORT

Neurofibroma the Benign Tumor of Oral Cavity: A Case Report

1˜ 2 3
Arnabjyoti Deva Sarma , Jibon Sharma , Moitrayee Devi

1
Faculty of Paramedical Science (Radiology), Assam down town University, Guwahati, Assam, India
2
Gauhati Medical College Hospital, Guwahati, Assam, India
3
Faculty of Paramedical Science (Microbiology), Assam down town University, Guwahati, Assam, India
˜
Corresponding author: Arnabjyoti Deva Sarma, Email: [email protected]
Article Chronicle: Received: 16/11/2021 Accepted: 31/12/2021 Published Online: 12/01/2022

Abstract
The malignant tumours of the tongue are far common than the benign ones. Papilloma, fibroma, adenoma, mucous
cyst, lymphangioma are the most commonly reported benign tumours. Neurofibroma of the tongue is also a rare
one. In this case report, lingual benign tumours and their distinctive histopathological features of a 22 years old
male patient has been reported with.The chief complaint of swelling in the tongue since one year. Incision biopsy was
done.Histopathology showed spindle cells with thin wavy nuclei along with fine collagen fibrils. Histopathological and
immunohistochemical studies confirmed the diagnosis of a myxoid predominant intramural solitary neurofibroma.The
purpose of this case report is to apprehendthe uncommon presentation of neurofibroma and to document the successful
management of such a lesion using an intraoral approach.
Keywords: Neurofibroma, Tongue, Histopathological, Radiology, Oncology

1. INTRODUCTION the swelling had been present for the last one year. The
Cases of Neurofibromas are very rare in head and neck swelling was asymptomatic, did not interfere with swal-
region. Up to 10% of these lesions are associated with lowing, speech or movements of the tongue. No history of
neurofibromatosis, an autosomal dominant disorder. Neu- altered sensation and any ulceration or discharge from the
rofibroma can be of three subtypes- localized, diffuse and swelling. Patient was conscious, co-operative with moderate
plexiform1; 2. Plexiform neurofibroma is least common and built, normal gait and posture. There were no positive signs
is path gnomic of von Recklinghausen disease, seen in 17- of pallor, icterus, cyanosis or clubbing. No systemic abnor-
30% of patient, caused by mutation of NF1 gene in chro- mality was detected. A dome shaped swelling was seen
mosome. Oral manifestations are described in only 4-7% on the left dorsal aspect in the posterior part of tongue.
of patient, and tongue is the most common site1; 3; 4. It measured approximately 25x30mm. The surface of the
Approximately 5-10% of plexiform neurofibroma under- swelling appeared normal with unaltered filiform and fungi
goes malignant transformation and their rate of growth is form papillae. No pulsation was found. There was no
inversely proportional to age. The growth of plexiform restriction of the tongue movements. No abnormality was
neurofibroma is usually ill-defined, and there is a risk of detected in any other soft tissue components of the oral cav-
recurrence(2; 4; 5; 6). Here, we present a case of an isolated ity including the palate, buccal mucosa, floor of the mouth
plexiform neurofibroma of the bottom of tongue. and lips etc. Fine Needle Aspiration Cytology (FNAC) of
the lesion was done.
2. CASE
A 22 year old male patient reported with the chief complaint
of painless swelling in the tongue since one year. Patient
had visited a dentist for the treatment of the swelling in
the tongue a few months back. The patient revealed that

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AMRIT, 1(1), 2022, 61-63 ISSN

and tumors during this region may cause an upper air-

way obstruction(13). Respiratory failure and autopsy re-
vealed laryngeal sub mucous plexiform neurofibromatosis
nodules as well as extensive plexiform neurofibromas involv-
ing the vagal, recurrent laryngeal and phrenic nerves. We
didn’t encounter with any lesion within the upper airway in
the present case. Differential diagnosis of such a tongue
mass in childhood must include neurofibroma, shwanno-
mas(neurilemoma), lymphangioma(14), cavernous heman-
gioma, hematoma, teratoma(15)lipoma(16),myofibroma
and myofibromatosis(17),leiomyoma(18),cyst-adenoma(19),
pyogenicgranuloma(20), nerve sheath myxoma(21), congen-
ital granular cell tumor(22), and cystic lesions such as
mucoid cysts(23), dermoid cysts(24), and cysts of foregut
origin(25).
Figure 1: Ultrasound showing hypo echoic lesion on the dor-
sal aspect of tongue on the left side

Frank blood was obtained on aspiration. On ultrasound,

a well-defined hypo echoic lesion measuring 26x28 mm was
seen on the dorsal aspect of tongue on the left side(Figure
1). The lesion was seen reaching up to the midline. On
Color Doppler study, no internal vascularity was noted. In-
cision biopsy was done and the stained sections revealed le-
sion tissue composed of delicate spindle cells with thin wavy
nuclei along with fine collagen fibrils. Myxoid and cellular
areas were seen. Mast cells were scattered throughout the
lesion. The patient was given empirical treatment with oral
propanolo and local sclerosant (phenol) injection. However, Figure 2: Histopathology shows the tissues of Neurofibroma
there was no decrease in size of the lesion so patient was
advised for surgical treatment. Patient did not agree for The treatment of such lesions is usually surgical and there-
the surgery and never reported back. fore the diagnosis can only be confirmed after histologi-
cal examination(Figure 2). It is important to differenti-
3. DISCUSSION
ate a neurofibroma from a shwannomas histopathologicaly;
Neurofibromas of the large nerves, which appear clinically since the Von Recklinghausen’s neurofibromatosis associ-
as soft, drooping and doughy masses[7], are benign neo- ated with neurofibroma has greater potential for malig-
plasm’s composed of neuritis, Schwann’s cells, and fibrob- nant transformation (5-16% is reported)(26). Neurofibro-
lasts within a collagenous or myxoid matrix(7). In con- mas have extensive vascularity and tend to bleed during
trast to shwannomas, they are nonencapsulated and engulf surgery. Early diagnosis of Neurofibroma is extremely im-
the nerve of origin. Plexiform neurofibromas, forming tor- portant and regular follow up of these cases is essential to
tuous cords along the segments and branches of a nerve detect recurrences.
with a tendency to grow centripetally, are poorly circum-
scribed tumors(7). This tumor is claimed to be indicative 4. CONCLUSION
of VRD albeit it’s going to be the sole manifestation of the The diagnosis of neurofibroma was confirmed by histopatho-
disease(8; 9). Neurofibromas, usually related to VRD(Von logical evaluation and immunohistochemicalstudies. Lo-
Recklinghausen’s Disease), are generally encountered as calized (solitary) neurofibromas most often occur as spo-
multiple lesions, and sometime it may occur as a solitary radic lesions, however, diagnosis of a solitary neurofibroma
tumor, as in the case. However, the plexiform neurofibroma prompts clinical evaluation to exclude the remote possibil-
is never encountered in type-2 and rapid climb of a plexi- ity of neurofibromatosis. The purpose of this case report is
form neurofibroma usually suggests transformation into a to apprehend the uncommon presentation of neurofibroma
neurofibrosarcoma [7]. Despite their occurrence within the and to document the successful management criteria of such
head and neck region, neural sheath tumors are rarely seen a lesion using an intraoral approach.
within the mouth. Only 4-7% of patients affected by neu-
rofibromatosis display oral manifestations(10). The mobile Conflict of Interest Authors do not have any conflict of
tongue is the most commonly involved site followed by buc- interest.
cal mucosa, floor of the mouth, palate, lips and gingival(11).
Reportavailable on occurrence of a series of neural tu- Acknowledgments We acknowledge the help and facil-
mors of the tongue in which there were two neurofibro- ities received from the Assam downtown University and
mas, and one malignant and five benign shwannomas(12). Ayursundra Hospital, Guwahati to carry out this work.
The base of the tongue may be a relatively rare location

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