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ABSTRACT : Fibrosarcoma is malignant mesenchymal neoplasm of the fibroblasts that rarely affects the oral cavity proper and can cause local recurrences or metastasis. It constitutes about 1% of all the malignancies affecting the human race. This article presents one such rare case of fibrosarcoma originating in the mandible in a 24 year old man.

1 2 3 4Arti S. Sachdev, Md. Asdullah, Arun Sachdev and Mohd. Naeem1 2Senior lecturer, Post Graduate Student, Department of Oral Medicine & Radiology, Career Post Graduate Institute of Dental Sciences & Hospital, Lucknow.3Senior Consultant, Dept of Medicine ; Vivekanand Polyclinic and Institute of Medical Sciences, Lucknow

INTRODUCTION : Fibrosarcoma is a malignant neoplasm

of the fibroblastic origin.1 Fibrosarcoma of head and neck

area represents 5% of all malignant intraosseous tumors. It

can occur in any location but mainly affects long bone

particularly, and its occurrence in craniofacial region is about

15%, mandible being the most common site. Although

fibrosarcoma has been reported in all groups, it is most

commonly seen in the 3rd and 6th decades of life.2

Fibrosarcoma may arise as a primary tumor in any part of the

jaws and may be classified as of either peripheral (periosteal)

or central (endosteal) type.3 Secondary fibrosarcoma of the

bone may be associated with fibrous dysplasia, Paget's

disease, bone infarct or cyst, and/or osteomyelitis; it may also

occur as a malignant transformation of giant-cell tumor of the

bone or be induced by prior irradiation.4

Clinically, in the oral cavity the major symptoms are pain,

swelling, paraesthesia, loosening of teeth and ulceration of

the overlying mucosa. 5 Radiological imaging of

fibrosarcomas reveals radiolucent lesions with a geographical

moth-eaten or permeative pattern of bone destruction.6

Fibrosarcomas are graded from low to high malignancy after

the FNCLCC grading system, depending on the number of

mitotic figures, tumour differentiation and the presence of

tumour-necrosis.7 The treatment of choice is radical surgery;

radiation therapy and chemotherapy can be used in inoperable

cases.8 The prognosis is highly dependent on the tumour-

grading and the success of complete resection. This paper

describes a case of fibrosarcoma originating in the mandible

of a young male.

CASE REPORT

A 25 -year-old male patient reported to the department with

the complaint of swelling in the lower front chin region since

2 months. The swelling was initially small pea sized and

gradually increasing in size. Patient consulted a local

practitioner who prescribed medication for a week. The

swelling did not regress following medication. The swelling

gradually increased to attain the present size and was

associated with pain on palpation. There was no associated

history of difficulty in speech & mastication , loss of appetite

with weight loss and fever. Patient did not give any history of

systemic illness or trauma to the head & neck region. The

patient was a labour by profession. There was no significant

contributing family history. The patient gave a history of

tobacco chewing & smoking since 7 years.

Extraorally, there was a diffuse irregular swelling in the lower

1/3 rd region of the face measuring approximately 8 x 4 cm

extending from the right angle of the mouth across the chin to

1 cm away from the left corner of the mouth. Inferiorly on the

right side the swelling extended 1 cm below the lower border

of mandible, while on the left side it extended 3 cms below the

lower border of mandible. The swelling was more prominent

on the left side as compared to the right side. (Fig 1)The

margins were diffuse and the overlying surface was lobulated.

Skin over the swelling was smooth and stretched with a

fibrosed sinus in the right parasymphyseal region. The

swelling was firm and fixed to the underlying structures.

Bimanual palpation revealed enlarged right and left

submandibular lymph nodes. The nodes were non tender,

hard in consistency and fixed to the underlying structure.

FIBROSARCOMA OF THE MANDIBLE - A CASE REPORT

Journal of Dental Sciences

University

Key Words: Fibrosarcoma, Mandible, Antoni Cells

Source of support : NilConflict of interest : None

CaseReport

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University J Dent Scie 2015; 1(2) : 74-76

Fig-1 Fig- 2

Mouth-opening was reduced to 26 mm. Intraorally there was

a diffuse swelling measuring approximately 8 x 2 cm,

extending from distal aspect of 36 to distal aspect of 45. (Fig

2) On the buccal aspect the swelling extended from the

marginal gingiva obliterating the buccal vestibule and on the

lingual aspect it extended from the marginal gingiva

obliterating the lingual vestibule. Mucosa over the swelling

was pale and covered with pseudomembranous slough. The

surface over the swelling was lobulated with ill defined

margins. The swelling was non tender and firm in

consistency. It was fixed to the underlying bone associated

with slight buccal expansion of the bone i.r.t 34 35 and 36.

IOPA i.r.t. 31 32 41 and 42 revealed irregular loss of lamina

dura, bone loss and diffuse radiolucency in the periapical

region of 31 32 41 and 42. (Fig 3)There was alteration in

trabeculation of the alveolar bone. The mandibular occlusal

crossectional view showed irregular loss of lamina dura and

diffuse radiolucency in the periapical region i.r.t. 31 32 41 and

42 with sunray pattern of bone at the lingual cortical margin.

(Fig 4)The orthopantomograph (OPG) revealed generalized

loss of lamina dura in the mandibular arch. A diffuse

radiolucency was seen in the periapical region of 31 32 41 and

42. The borders of the radiolucency were ragged and non

corticated. Also multiple radiolucencies were seen in the

periapical region of 43 44 45 and 46. (Fig 5)

Fig -3 Fig-4

Fig-5

The intraoral mass was subjected to incisional biopsy. (Fig 6)

Fig-6

Fig-7

The section revealed a highly cellular section with scant collagen

fibres. Antoni type-A cells with elongated or spindle shaped

nuclei were seen arranged in chords. Antoni type-B cells with

highly pleomorphic, deeply basophilic nucleus were seen in

clusters. Large round cells with eosinophilic cytoplasm and

eccentrically placed nucleus were seen in group in few areas of

the section. (Fig 7). Correlating the clinical, radiological and

histopathological findings the diagnosis of Fibrosarcoma of the

mandible was made and the patient was referred for surgical

excision. However, after a follow up of 2 years, the patient

succumbed to the lesion.

DISCUSSION : Fibrosarcoma is a malignant tumor that arises

from the fibroblasts. Malignancies of the fibroblasts are rare in

the oral and oropharyngeal region, but fibrosarcoma is

nevertheless, the most common mesenchymal cancer of the

region, representing more than half of all sarcomas.8 The exact

cause of fibrosarcomas not entirely understood; however,

studies indicated that genetic alterations may play a role.

Fibrosarcoma can arise in soft tissues or within bone. Intra-

osseous fibrosarcomas may develop endosteally or possibly

periosteally, the latter affecting bone by spread from adjacent

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University J Dent Scie 2015; 1(2) : 74-76

soft tissue.9 Occasionally, the lesion erodes the roots of

involved teeth.

Generally the tumor develops with equal frequency in males

and females. The symptoms of fibrosarcoma vary depending

on size, location and spread of the tumor. Fibrosarcoma of the

oral cavity most often manifests as a clinically innocuous,

lobulated, sessile, painless and nonhemorrhagic submucosal

mass of normal coloration. Pain and paresthesia are usually

late symptoms indicating nerve involvement.2

Radiographically, fibrosarcoma often appears as a purely

osteolytic lesion without calcification and with poorly

defined, irregular margins if it has arouse intraosseously.

There is usually destruction of the cortical plates without

expansion.10 The absence of tumoural calcification or

ossification can be of importance in differentiating

fibrosarcomas from other malignancies such as

chondrosarcomas and osteosarcomas. Histopathologically ,

fibrosarcoma has been characterized by uniform spindle cells

distributed in interlacing fascicles with herring bone growth

pattern. The differential diagnosis for fibrosarcomas include

all spindle cell tumors, and only careful examinations of

multiple sections and special stains as well as

immunohistochemical analysis will permit a correct

diagnosis.11

The treatment of choice is surgical resection with a wide

margin. The need for adjuvant radiotherapy and/or

chemotherapy is still unclear but there is normally an

indication in high grade tumours because these tumours may

present with subclinical or microscopic metastases at the time

of diagnosis. The need for prophylactic neck dissection is

controversially discussed and it is not performed in all

cases.12 The overall survival rate at 10 years may vary from

21.8% to 83%, and clinical stage, histological grade of

malignancy, and local recurrences are the most important

prognostic factors.

CONCLUSION : In recent years, the fibrosarcoma of head

and neck area has been increasingly reported but still there are

paucity of reports of fibrosarcoma of head and neck region.

This rare tumor, which generally affects the long bones and

deep soft tissue, must be differentiated from other similarly

rare forms of sarcoma that may involve the oral cavity.

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CORRESPONDANCE :

Dr. Md. Asdullah

PG Student

Department of Oral Medicine & Radiology

Career Post Graduate Institute of Dental Sciences & Hospital

Lucknow

E-mail: drasadalig123@gmail.com

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