JIAOMR

10.5005/jp-journals-10011-1145
CASE REPORT Ewing’s Sarcoma of the Mandible: A Rare Case Report and Review of Literature

Ewing’s Sarcoma of the Mandible: A Rare Case

Report and Review of Literature
1
Sanki Reddy Shailaja, 2Marthala Manjula, 3Manika, 4Chappidi Vani, 5Ainampudi Bhargavi Krishna
6
Gannepalli Asha Lata
1
Senior Lecturer, Department of Oral Medicine and Radiology, Panineeya Dental College and Research Center
Hyderabad, Andhra Pradesh, India
2
Professor and Head, Department of Oral Medicine and Radiology, Government Dental College and Hospital
Hyderabad, Andhra Pradesh, India
3
Reader, Department of Oral Medicine and Radiology, BK Patil Dental College, Hyderabad, Andhra Pradesh, India
4
Reader, Department of Oral Medicine and Radiology, Sri Sai Dental College, Vikarabad, Andhra Pradesh, India
5
Reader, Department of Oral Pathology, Panineeya Dental College and Research Center
Hyderabad, Andhra Pradesh, India
6
Associate Professor, Department of Oral Pathology, Panineeya Dental College and Research Center
Hyderabad, Andhra Pradesh, India

Correspondence: Sanki Reddy Shailaja, Senior Lecturer, Department of Oral Medicine and Radiology, Panineeya Mahavidyalaya
Institute of Dental Sciences and Research Center, Road No 5, Kamala Nagar, Dilsukhnagar, Hyderabad-500060, Andhra Pradesh
India, e-mail: drsrshailaja@gmail.com

ABSTRACT

Ewing’s sarcoma is a small, round and blue cell malignancy that most commonly arises in the skeleton of adolescents and young adults.
Although it may appear in any bone, it is more common in the axial skeleton, rarely involving the jaws (1-2% incidence mostly in the
mandible). In this article, we are reporting a rare case of Ewing’s sarcoma of mandible in an 18-year-old female patient with the typical
radiographic appearance of spiculated bone formation.
Keywords: Ewing’s sarcoma, Small round cell tumor, ES/PNET.

INTRODUCTION midline. Overlying skin is stretched and shiny with venous

prominence (Fig. 1). It is soft to hard in consistency. Intraoral
Ewing’s sarcoma was first described by James Ewing in 1920
examination revealed buccal and lingual cortical plate expansion
as a diffuse endothelioma of bone. Ewing’s sarcoma constitutes
and mobility in relation to 45, 46, 47 and 48. There was lingually
6 to 8% of all primary malignant tumors and represents the
and buccally displaced 47 and 48 respectively (Fig. 2).
third most common osseous neoplasm after osteosarcoma and
chondrosarcoma. 1 Ewing’s sarcoma is genetically and
histologically distinctive small round cell sarcoma of bone, and
it constitutes 1% of all malignant tumors in children.2 It is a
notoriously aggressive and destructive malignancy of bone
arising from marrow mesenchymal stem cells. Since then, it
has been documented as a distinct malignancy of primitive
mesenchymal stem cells that have undergone a unique reciprocal
translocation of chromosomes 11 and 22.3

CASE REPORT

An 18-year-old female patient reported to the Department of

Oral Medicine and Radiology (Govt Dental College and
Hospital, Afzalgunj, Hyderabad) with swelling in right lower
side of the jaw since one year. Initially, swelling was small and
progressive rapid growth was observed in last 6 months, later
paresthesia of right lower lip was noticed. Extraoral examination
revealed diffuse swelling present on the right side of angle and
body of mandible extending into submandibular region crossing Fig. 1: Extraoral photograph showing large submandibular swelling

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Sanki Reddy Shailaja et al
Orthopantomograph (OPG) and lateral skull radiographs
showed mixed radiolucent-radiopaque lesion with ill-defined
ragged borders extending from 45 to posterior border of ramus
of mandible, with spiculated bone formation at the right angle
of mandible. OPG also showed root resorption of 48 (Figs 3
and 4).
Computed tomography (CT) revealed osteolytic and
osteoblastic lesions involving ramus and angle of right mandible
with large soft tissue component and new bone formation.
Periosteal reaction observed in ramus of right mandible,
suggestive of osteosarcoma (Fig. 5). Skeletal scintigraphy given
an impression of bony swelling of right mandible with
osteoblastic components and possible necrosis. No distant
skeletal metastasis seen.
Histopathological examination showed fragments of a
cellular lesion along with fibrous tissue and bone. The
lesion is comprised of monomorphous round cells with
round to oval nuclei having scanty cytoplasm (Fig. 6). The
nuclei showed dispersed chromatin, inconspicuous nucleoli,
and mitotic activity is seen. The lesion also shows
prominent, collapsed thin-walled vessels.
Immunohistochemistry showed strong membrane
positivity noted in the tumor cells for CD99 and negative
for LCA. Features are consistent with ES/PNET (Ewing’s
peripheral neuroectodermal tumor) mandible.
Fig. 2: Intraoral photograph showing displacement of 47 and 48
lingually and buccally respectively
Fig. 3: Orthopantomogram
272
Fig. 4: Lateral skull radiograph showing spiculated bone
DISCUSSION
Ewing’s sarcoma belongs to the Ewing sarcoma family of tumors
(ESFT), which comprises a group of small round cell neoplasms
of neuroectodermal origin and includes the primitive
neuroectodermal tumors. ESFT may arise in osseous or
nonosseous sites and in multiple locations, including the soft
tissues anywhere in the body, such as the paravertebral and
thoracic areas.4 There is considerable clinical and histologic
overlap between this tumor and primitive neuroectodermal
tumor. Most investigators now believe that ES and PNET are
different morphological expression of one tumor type.5
Ewing’s Sarcoma can affect any bone but the most common
sites are the lower extremity (45%), followed by the pelvis
(20%), upper extremity (13%), axial skeleton and ribs (13%)
and face (2%).6 Facial skeletal quite rare with mandible being
the most commonly affected bone.2 Approximately 90% of the
reported cases occurring in the mandible have been primary
lesions and 10% have been metastases.7
It is mostly seen in patients younger than 20 years (80%).
The peak age of occurrence is in the teenage years (50%).
It practically never occurs in black individuals. Young men are
affected slightly more often than young women (1.4:1).3
ES clinically usually presents with the well-known
inflammatory complex of fever, pain, swelling, paresthesia and
leucocytosis. History of fever is not a constant feature. Pain
varies from moderate to very severe, is often intermittent in
character and is more marked at night. Pain in other parts of the
skeleton is the earliest indication of metastasis. Enlargement of
the nearest draining lymph nodes is often present.8
The most frequent radiographic findings described were
permeative bone destruction and the presence of an adjacent
soft tissue mass. ES of the jaws presents similar radiographic
features to involvement of the peripheral skeleton. These
include: (1) A permeative destructive pattern characteristic of
the small round cell tumor, (2) periosteal reaction was seen in
over half of the cases. The laminated type of periosteal reaction
is difficult to demonstrate in the jaws because of the complex
anatomy, (3) adjacent soft tissue involvement is a very common
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Ewing’s Sarcoma of the Mandible: A Rare Case Report and Review of Literature
Fig. 5: Coronal view showing large soft tissue component
Fig. 6: Histopathology picture showing monomorphous round cells
with round to oval nuclei having scanty cytoplasm
finding in ES. Dental elements were involved in 75% of the
cases in the form of displacement of the tooth follicle or erupted
permanent teeth.9
Some reports indicate that the radiological features of ES
in the mandible are characterized by a periosteal reaction in the
form of sunray spicules, whereas others have described an onion
skin pattern of periosteal reaction in ES in the jaws.7
The radiologic differential interpretation of ES of the
mandible consists of osteogenic sarcoma, neuroblastoma,
lymphosarcoma, histiocytosis X, rhabdomyosarcoma,
osteomyelitis and metastatic carcinoma. The presence of a large
soft tissue mass aided differentiation of Ewing’s tumor from
osteomyelitis and eosinophilic granuloma. The age of the patient
ruled out the possibility of neuroblastoma, which is common in
less than 5 years age group. However, radiology is not totally
reliable guide to diagnosis and histopathological examination
is necessary to confirm the nature of the tumor.10
The differential diagnosis of ES is one of exclusions because
it has no specific markers. All small, round, blue cell tumors of
childhood, such as primary bone sarcomas, rhabdo-
myosarcomas, lymphomas, neuroblastomas and primitive
neuroectodermal tumors should be considered.11
The diagnosis is established by biopsy, in which the tumor
is seen as layers of small round cells, similar to lymphocytes
but larger. Mitotic cells are rare, intercellular stroma is scarce
Journal of Indian Academy of Oral Medicine and Radiology, July-September 2011;23(3):271-274
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and a large portion of the tumor may be necrotic. Tumor cells
placed around a clear central area forming rosettes may be seen,
resembling Homer-Wright rosettes, typical of neuroblastomas.
Intracytoplasmic glycogen is a definitive aspect, but not
pathognomic because it is also present in other tumor cells, such
as osteosarcomas, rhabdomyosarcomas and neuroblastomas.12
Recent immunoperoxidase and cytogenic studies indicate
that PNET and ES are the same entity showing varying degrees
of neuroectodermal differentiaton and they are categorized into
a group known as the Ewing family of tumors. CD 99/MIC2 is
a cell surface glycoprotein found in virtually all ES and PNET.
But it is also detected in other small round cell tumors, such as
T-lymphoblastic lymphoma, poorly differentiated synovial
sarcoma, small cell osteosarcoma, rhabdomyosarcoma,
desmoplastic round cell tumor, small cell carcinoma and Merkel
cell carcinoma and it should be used as part of a panel of
immunostains, given its lack of complete specificity.13
More than 90% of cases show a characteristic translocation
t (11; 22) (q24; q12) resulting in the fusion of the EWS and
FLI-1 genes. This gene rearrangement causes a fusion product
which functions as an oncogenic aberrant transcription factor
with structural variability and potentially prognostic impact.14
The prognosis of ES is poor because of multiple metastases
most commonly to the bone, lung, lymph node and liver, which
may occur within a few months after the onset of the tumor.15
Poor prognostic factors are patients below 10 years of age, pelvic
lesions, presence of systemic symptoms, large tumor volume
(> 200 ml), high mitotic rate, filigree pattern in histopathological
sections and poor response to chemotherapy. Ewing’s sarcoma
of the mandible has got better prognosis than long bones since
facial sites are diagnosed earlier.2
Between 20 and 25% of the patients are diagnosed with
metastatic disease (10% lung, 10% bone/bone marrow, 5%
combination or others). A chest CT scan is required to rule out
lung or pleural metastases. The assessment for bone and bone
marrow metastasis is to include 99m Tc bone scintigraphy, to
detect osseous metastasis, and light microscopical examination
of bone marrow aspirates and biopsies taken at sites distant
from the tumor. Positron-emission tomography (PET) scanning
for bone metastases and PCR techniques to investigate for bone
marrow metastases are sensitive methods currently under
evaluation.16
With surgery or radiotherapy alone, 5-year survival is
< 10%. With treatment in current multimodality trails including
chemotherapy, survival is 60 to 70% in localized and 20 to
40% in metastatic disease. Bone metastasis confers a poorer
outcome than lung/pleura metastases (less than 20% compared
with 20 to 40% 5-year survival). All current trails employ 3 to
6 cycles of initial chemotherapy after biopsy, followed by local
therapy and another 6 to 10 cycles of chemotherapy usually
applied at 3 weeks intervals. Treatment duration is thus 8 to
12 months. Agents considered most active include doxorubicin,
cyclophosphamide, ifosfamide, vincristine, dactinomycin and
etoposide. Protocols that have proved to be most effective
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Sanki Reddy Shailaja et al
include atleast one alkylating agent (ifosfamide or
cyclophosphamide ) and doxorubicin. Radiotherapy is applied
at doses of 40 to 45 Gy for macroscopic disease. Most relapses
occur in the first 3 years of follow-up; late relapses have rarely
been observed even after 15 years or longer. Follow-up intervals
should be 2 to 3 months during the first 3 years, 6 months until
5 years and atleast once yearly thereafter.16
CONCLUSION
In general, Ewing’s sarcoma is a rare malignancy that may affect
the facial bones of young individuals. Even with an isolated
area of ES, the risk of metastasis is so great that it may warrant
multiple therapy modalities. In case of suspected cases, an
evaluation of the lesion should be carried out using plain films,
CT, MRI, bone scan and biopsy. After treatment, it is mandatory
to provide suitable prosthesis, so that these young patients lead
a quality life.
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