fibrosarcoma of the mandible - a case … j dent scie 2015; 1(2) : 74-76 abstract : fibrosarcoma is...
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University J Dent Scie 2015; 1(2) : 74-76
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.
REFRENCES
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mandible,” Medicina Oral, Patolog´ýa Oral y Cirug´ýa
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fibroblasts,” Cancer, Vol. 1, No. 1, pp. 30–63, 1948.
3. Mcleod JJ, Dahlin Dc and Ivins JC: Fibrosarcoma of bone.
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11. Lukinmaa PL, Hietanen J, Swan H, Ylipaavalniemi P,
Perkki K. Maxillary fibrosarcoma with extracellular
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Takagi M et al. Sarcomas of the oral and maxillofacial
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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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