Case Report

Osteosarcoma of the jaw: Primary versus

secondary ‑ A report of two cases
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ABSTRACT
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Manisha Ahuja,
Osteosarcoma (OS) is the most common primary malignancy of bone excluding hematological malignancies. Most common sites Shramana
of tumor are long bones of extremities. OS of the jaw are extremely rare with mandible being more commonly after than maxilla. Mandal,
Hereby, we present two cases of OS of jaw with one patient being male and other female. Varuna Mallya,
Nita Khurana,
Ravi Meher1,
KEY WORDS: Cemento‑ossifying fibroma, jaw, osteosarcoma, primary, secondary Kishore Singh2

Departments of
Pathology, 1ENT
INTRODUCTION Patients with OS of the jaw present with symptoms and 2Radiotherapy,
like painful swelling in the area with loosening Maulana Azad Medical
Osteosarcoma (OS) is the most common primary of teeth. Symptoms such as paresthesia, nasal College, New Delhi,
India
malignant tumor of the bone. It is defined as a obstruction, and ophthalmic complications such
malignant tumor of the bone in which malignant as proptosis can also occur.[1] For correspondence:
mesenchymal tumor cells have the ability to Dr. Varuna Mallya,
produce osteoid or immature bone. Two cases of OS of the jaw are being presented here. Room No. 269,
Pathology Block,
One, a case of a primary OS, and the second case
Department of
OS accounts for approximately 20% of all primary of secondary OS arising in a known case of COF. Pathology, Maulana
sarcomas of the bone, excluding multiple myeloma Azad Medical College,
and other hematopoietic neoplasms. The most CASE REPORTS New Delhi ‑ 110 002,
common sites of involvement are long bones of India.
E‑mail: varunamallya@
extremities such as the distal femur, proximal tibia, Case summary 1
gmail.com
and humerus. Only 5% of cases occur in the jaw. A  35‑year‑old male presented with complaints of
The mandible is more commonly affected than the swelling over the right cheek for 4 months.
maxilla. The age distribution is bimodal,[1] with
the first major peak occurring during the second On examination, a 5 cm × 4 cm swelling was
decade of life, and the second, much smaller, peak is found over the right cheek [Figure 1a]. The
observed in patients older than age 50 years where it mouth opening was adequate with slight
occurs in the background of preexisting bony lesion. redness of the right eye. The swelling was
firm‑to‑hard in consistency, nonmobile, and
OSs can be primary or secondary.[2] Primary OS painless. The overlying skin was normal.
occurs in a normal bone, whereas Secondary OS
arises in a bone that is altered either due to prior A computed tomography (CT) scan of the right
radiation, infarction, or a preexisting disease maxilla was performed which revealed a soft‑tissue Submitted: 19‑Dec‑2021
such as Paget’s disease or ossifying fibromas. mass in the right maxillary sinus causing erosion Accepted in revised
Cemento‑ossifying fibroma (COF) is a benign of the posterolateral wall and extending into form: 13‑Jan‑2022
fibro‑osseous tumor that is composed of cellular pterygomandibular fossa causing erosion of Published: 25-Apr-2023
stroma with mineralized matrix. The tumor is a the medial wall of the left maxillary sinus, the
Access this article online
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slowly progressive enlargement of the affected This is an open access journal, and articles are distributed under the terms of the cancerjournal
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DOI: 10.4103/jcrt.jcrt_2295_21

site. The tumor is mostly seen in females. Rarely, Quick Response Code:
long as appropriate credit is given and the new creations are licensed under the
there can be the development of OS, often low grade identical terms.
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Cite this article as: Ahuja M, Mandal S, Mallya V, Khurana N, Meher R, Singh K. Osteosarcoma of the jaw: Primary versus
secondary ‑ A report of two cases. J Can Res Ther 2023;19:2086-9.

2086 © 2023 Journal of Cancer Research and Therapeutics | Published by Wolters Kluwer - Medknow
 Ahuja, et al.: OS‑jaw
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a b
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c d
Figure 1: (a) Clinical photograph showing a swelling in the right maxillary
region, (b) computed tomography scan showing a lytic lesion in the right
maxilla with cortical destruction, (c) photomicrograph showing tumor
cells laying down osteoid (H and E, ×100), and (d) photomicrograph
showing atypical spindle cells within the osteoid (H and E, ×400)
anterior wall of the right maxillary sinus with extension to
the right cheek with the erosion of floor of orbit with minimal
infraorbital extension. The scan was likely suggestive of
neoplastic etiology [Figure 1b].
The patient underwent resection, and on microscopic
examination, tumor tissue was found composed
of spindle‑shaped cells showing moderate to marked
pleomorphism. Few tumors giant cells were identified
along with areas of lacy osteoid laid down by tumor cells
[Figure 1c and d]. Based on the histomorphological and clinical
findings, a diagnosis of OS was rendered.
Since the patient had no evidence of metastasis and the
margins were free. The patient was not given any postoperative
radiotherapy or chemotherapy, and he is currently on follow‑up
and disease‑free.
Case summary 2
A 25‑year‑old female presented with swelling over the left
mandibular region for 3 months [Figure 2a].
On examination
A 10 cm × 8 cm swelling was present over the left mandibular
region. Swelling was firm‑to‑hard, nontender, and fixed. The
overlying skin was normal.
The patient was subjected to radiological investigations. CT
scan revealed a lytic lesion with associated cortical breach
involving the body and angle of the left mandible. The lesion
Journal of Cancer Research and Therapeutics - Volume 19 - Issue 7 - October-December 2023
a b
c d
Figure 2: (a) Clinical photograph showing a swelling over the left
mandible, (b) computed tomography scan showing a lytic lesion with
cortical breach involving the bone and angle of the mandible, (c)
intraoperative assessment showing a nodular mass arising from the
mandible, and (d) gross photograph showing the lesion in the mandible
was seen involving the left lower second premolar, first
and second molar teeth with large associated soft‑tissue
components infiltrating into the adjacent masseter muscle
and enlarged submandibular lymph nodes. Findings were
suggestive of malignant jaw tumor [Figure 2b].
Now, a hemimandibulectomy was performed which revealed
a partially encapsulated tumor [Figure 2c and d]. The patient
had a history of similar swelling at the same site 2 years back.
The microscopy then revealed masses of cementum‑like tissue
surrounded by a hypercellular stroma, there was no evidence
of atypia or mitosis, and a diagnosis of COF was rendered
then [Figure 3a].
Microscopic examination of the present lesion exhibited
tumor tissue composed of proliferating spindle cells. The
stroma showed deposition of osteoid with infiltration by
tumor cells [Figure 3b]. Few mitotic figures were seen.
A diagnosis of low‑grade OS (LOS) arising in a case of COF
was made.
The patient had no evidence of metastasis and was on close
follow‑up. The patient has no symptoms of recurrence and at
present disease‑free.
DISCUSSION
OS is the most common malignant bone tumor and is a relatively
rare disease in the orofacial region.[3,4] OS of craniofacial bones
accounts for 6% to 10% of all cases of OSs.[5,6] The mandible
and the maxilla are the most frequently involved bones,
representing 3% and 1.5% of all cases of OSs, respectively.
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 Ahuja, et al.: OS‑jaw
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Figure 3: (a) Photomicrograph showing cementum surrounded by
hypercellular stroma (H and E, ×100), (b) photomicrograph showing
osteoid laid down by tumor cells, some of which are entrapped in
osteoid (H and E, ×400)
Approximately 80% of the cases of the jaw are reported in patients
older than 20 years of age. Although the age range is wide, rare
cases are seen which have been reported in children <10 years
of age. The peak incidence of craniofacial OS is during the third
and fourth decades of life with no sex predilection. Here, the age
of the patients was in the third and fourth decade with one case
arising in male and other in female.[3,7]
Primary OS occurs in normal bones, whereas secondary OS
occurs in an already altered bone affected by irradiation,
infarction, or diseases such as ossifying fibroma or Paget’s
disease. Herein, we had two cases of OS, one being primary
and the second one arising in preexisting COF.[1,3,4]
Similar studies have been conducted in the past in which
OSs originating from COF have been reported. Lee et al.[7]
presented a case of the secondary LOS arising from COF in
a 45‑year‑old female. The patient was treated with mass
excision multiple times over a period of 2 years and 8 months
and eventually she was diagnosed with LOS and underwent
wide excision, segmental mandibulectomy, and reconstruction
with fibula‑free flap. COFs are benign fibro‑osseous lesions
composed of fibrous stroma and bone. It is seen mostly in
females. They can be unilocular or multilocular and arise
mostly in the mandible. Transformation of COF to OS is
extremely rare with very few cases reported in the literature.
Although OS of the jaws is very aggressive, Lee et al.[7] reported
a case of low‑grade secondary OS arising in the jaw in the
case of COF. There was no metastasis, and the patient was
treated with resections with wide surgical margins. Our
second case also showed features of LOS and was treated with
hemimandibulectomy with wide surgical margins. No adjuvant
chemotherapy/radiotherapy was given, and the patient is
disease‑free after 4 years.
Conventional OS belongs to the category of the most
aggressive osseous neoplasms.[8] OSs of the craniofacial bones,
especially those of the mandible, tend to have a more indolent
course than other conventional OSs.[9] Satellite intramedullary
foci (“skip” metastases) are considered to be a major source
of local recurrence.[10] Metastases are less frequent than local
recurrence and occur in about one‑third of patients with
2088 Journal of Cancer Research and Therapeutics - Volume 19 - Issue 7 - October-December 2023
craniofacial OSs. They typically occur within 2 years of initial
treatment, predominantly in the lungs. Surgical treatment
with wide margins is the first choice of treatment for
jawbone OS. Although chemotherapy treatment for OSs helps
in the reduction of tumor size, chemotherapy for jawbone
OSs was a matter of discussion in the past.[11] At present, a
multimodal approach consisting of a combination of surgery,
chemotherapy and/or radiotherapy is strongly recommended,
and the prognosis has progressively improved over the
years.[2,9] The overall 5‑year survival rate for mandibular
and maxillary tumors after combined modality treatment is
approximately 75%.[10]
The patients treated for head‑and‑neck OSs must be followed
up every 2 months in the first 2 years after treatment, every
3 months during the 3rd year, and biannually for the rest of
their lives.[11]
OS jaw must be differentiated from other lesions such as central
giant cell granuloma, ossifying fibroma, fibrous dysplasia, and
other mesenchymal malignancies of the jaw. Pure osteolytic
OS can mimic undifferentiated pleomorphic sarcoma (UPS),
fibrosarcoma, or giant cell tumors. OS is differentiated from UPS
by the absence of storiform pattern/fascicles and the presence
of osteoid material. The absence of classic herringbone
pattern helps in distinguishing OS from fibrosarcoma. Since
OS is also giant cell‑rich, presence of osteoid material with
entrapped atypical cells helps in discriminating it from other
giant cell‑rich lesions OS occurring in the diaphysis should
be differentiated from Ewing’s sarcoma or lymphoma. [10]
Ewing’s sarcoma and lymphoma have a small round blue cell
morphology which is not seen in OS. Out of these, it is most
difficult to differentiate LOS from fibrous dysplasia as it occurs
as a diffuse lesion. The presence of invasive growth pattern and
mild atypia in LOS is the most helpful feature in differentiating
LOS from fibrous dysplasia.
Radiologically, LOS can be misinterpreted as a benign lesion. It
can mimic various lesions such as fibrous dysplasia, giant cell
tumor, nonossifying fibroma, and osteoblastoma.[10,11] LOS is
differentiated from these lesions on the basis that it shows the
presence of poor margination and cortical destruction with or
without extension into the soft tissue. Clinically, this needs to
be distinguished from Garre’s osteomyelitis and rarely Ewing’s
sarcoma. The former is characterized by periosteal new bone
formation in onion‑skin pattern, whereas the latter by new
bone that is laid parallel to the cortex.[12,13]
These cases are being presented for its rarity and to make
the pathologist aware of its unusual site. Early diagnosis and
surgical resection with wide margins are important to prevent
the spread and better prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient (s) has/have
 Ahuja, et al.: OS‑jaw
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initial s will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
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Nil.
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Conflicts of interest
There are no conflicts of interest.
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