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Correspondence

Parosteal osteosarcoma of mandible:

A rare case report
ABSTRACT Swati Gupta,
With the exception of multiple myelomas, osteosarcoma is the most frequently occurring primary malignant bone tumor with an Shilpa Parikh1,
overall incidence of 1:100,000/year. It has greatest predilection for the metaphyses, most frequently femur and tibia. However, Sumit Goel
osteosarcomas affecting the craniofacial bones are infrequent. Two main types: intramedullary and juxtacortical varieties are
Department of
seen. Juxtacortical variety is further subdivided into periosteal and parosteal variants. Due to its rarity, only 13 cases of parosteal Oral Medicine and
osteosarcoma have been reported till date. A 35‑year‑old male patient with affected postirradiated mandible is being reported as Radiology, Subharti
the 14th case of this kind with its unique benign presentation and less aggressive nature. Dental College, Swami
Vivekanand Subharti
University, Meerut,
KEY WORDS: Jaw, juxtacortical, mandible, osteosarcoma, parosteal, postradiation Uttar Pradesh,
1
Department of
Oral Medicine
and Radiology,
INTRODUCTION CASE REPORT Government Dental
College and Hospital,
Ahmedabad, Gujarat,
The term first used by Alexis Boyer in 1805, A 35‑year‑old male patient reported with a
India
“osteosarcoma” represents the most common complaint of pus discharge from the left side of
nonhematopoietic primary malignant bone the face for 9 months. Three months back, his left For correspondence:
tumor. In the head and neck region, they lower second premolar and molars exfoliated. He Dr. Swati Gupta,
account for only 6–10% of all osteosarcomas.[1] Department of
gave a history of surgery followed by radiotherapy
Oral Medicine and
Osteosarcomas of the jaw bones are very rare, for nasopharyngeal angiofibroma 18 years back and Radiology, Subharti
with an incidence of 0.7/million, and are more history of tuberculosis and extraction of upper left Dental College,
frequent in the mandible than in the maxilla. They posterior teeth 4 years back. Swami Vivekanand
usually present in the third and fourth decades of Subharti University,
life, almost a decade after their presentation in Subhartipuram,
On examination, there was a single diffuse, slightly NH‑58, Delhi
long bones.[2] Its induction is promoted by Rb and tender, and firm swelling anteriorly, discolored skin Haridwar
p53 inactivation localized to 17p13 and 13q14, and extraoral sinus opening posteriorly [Figure 1]. Bypass Road,
respectively. Cytogenetic abnormalities should No lymphadenopathy was present. Intraorally, Meerut ‑ 250 005,
also be checked in chromosomes 1, 6, 9, 10, 11, Uttar Pradesh, India.
a single 6 cm × 4 cm × 3 cm sized bluish red E‑mail: drswatig@
12, 14, and 15.[3]
pedunculated, slightly tender, lobulated, and rediffmail.com
firm irregular growth was present on the lower
Zarbo and Carlson have classified osteosarcomas by
left alveolar ridge with superficial ulceration
their site of origin into – conventional/intramedullary,
and indentations of lower teeth [Figure 2]. It was
juxtacortical (arising from periosteal surface),
protruding out from left corner of the mouth and
and extraskeletal osteosarcomas.[4] Juxtacortical
osteosarcoma (incidence of 0.07/100,000 pushing tongue to the opposite side. Both upper
population) has two clinicopathologic variants: and lower teeth in vicinity of the growth were
parosteal (low‑grade) and periosteal (high‑grade).[5] grade III mobile. Clinical differential diagnosis of
osteoradionecrosis in association with metastatic Access this article online
To date, only 28 cases of juxtacortical osteosarcomas tumor and osteosarcoma were made. Website: www.cancerjournal.net

have been reported of which 13 were parosteal type, DOI: 10.4103/0973-1482.176420

This is an open access article distributed under the terms of the Creative Commons
nine were periosteal, and six were of periosteal/ Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows others to remix,
PMID: ***

parosteal type.[6] The present case brings the total Quick Response Code:
tweak, and build upon the work non‑commercially, as long as the author is credited
number of parosteal osteosarcomas of jaws to 14, and the new creations are licensed under the identical terms.
a rarity in itself in the literature. For reprints contact: reprints@medknow.com

Cite this article as: Gupta S, Parikh S, Goel S. Parosteal osteosarcoma of mandible: A rare case report. J Can Res Ther
2018;14:471-4.

© 2016 Journal of Cancer Research and Therapeutics | Published by Wolters Kluwer - Medknow 471
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Gupta, et al.: Parosteal osteosarcoma of mandible
Panoramic radiograph [Figure 3] showed mixed radiolucent
radiopaque pattern in the lower left quadrant with
pathologic fracture. Hanging drop appearance of the
upper left canine was seen. The findings of computed
tomography were suggestive of malignant mass (irregular
heterogeneously enhancing mass) of 46 mm × 76 mm in the
left buccal mucosa, involving upper and lower gingivobuccal
sulcus with significant soft tissue component abutting
oral tongue and extending into buccinator space, root of
zygomatic arch, pterygopalatine fossa, and infratemporal
fossa [Figure 4]. There was erosion of the upper and lower
alveolus, posterior wall of right maxillary sinus. Enlarged
lymph nodes measured 17 mm × 16 mm in the left level IB.
Incisional biopsy from the growth was in favor of pyogenic
granuloma; however, based on clinical and radiographic
findings, left hemimandibulectomy was planned and
surgical specimen was then sent for histopathological
examination [Figure 5].
On histopathological examination, gross tumor mass of
size 6 cm × 6 cm × 4.5 cm arising from the surface of
the mandible showed proliferation of well‑differentiated
fibroblastic cells with osteoid. Pleomorphism was not
present. Mitosis present was 4–6/10 hpf at active area.
Figure  1: Extraoral photograph showing single diffuse swelling
anteriorly, discolored skin, and extraoral sinus opening posteriorly
Figure 3: Panoramic radiograph showing irregular destruction of bone
extending from 33 to 38 and pathologic fracture
472 Journal of Cancer Research and Therapeutics - Volume 14 - Issue 2 - January-March 2018
Atypical mitosis was absent [Figure 6]. All surgical margins
were free of tumor.
On the basis of clinical, radiological, histopathological features,
a diagnosis of parosteal osteosarcoma of the mandible was
made.
DISCUSSION
In 1949, Geschickter and Copeland were the first to
describe parosteal osteosarcomas. These are rare, low‑grade
juxtacortical variant of osteosarcoma, especially in the jaws,
representing 1.6% of all bony malignant tumors and up to
5% of all osteosarcomas. Only 13 cases of intraoral parosteal
osteosarcoma have been reported in the English literature. The
presenting patients varied from 13 to 68 years with the average
age being 38 years with equal sex predilection. The maxilla
Figure 2: Intraoral photograph of the growth on lower left alveolar ridge
which is irregular bluish red and approximately 6 cm × 4 cm × 3 cm in
size with pedunculated base and superficial ulceration
Figure 4: Computed tomography (coronal and axial sections) revealed
presence of 46 mm × 76 mm irregular heterogeneously enhancing mass
lesion seen in left buccal mucosa extending along the upper and lower
gingivobuccal sulcus. The lesion abutted left mylohyoid muscle and
displaced tongue to right side; and extended into buccinator space,
root of zygomatic arch, pterygopalatine fossa, and infratemporal fossa
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Gupta, et al.: Parosteal osteosarcoma of mandible
Figure  5: Postoperative extraoral and intraoral photograph of the
patient after left hemimandibulectomy
was more commonly affected with eight cases, whereas the
mandible was affected in five cases.[6,7] In the present case,
mandibular involvement was seen in a 35‑year‑old male
patient.
Well‑recognized risk factors include preexisting bone
abnormalities, hereditary retinoblastoma, Li–Fraumeni
syndrome, Rothmund–Thomson syndrome, and history of
radiation exposure,[8] which was seen 18 years back in the
present case. Osteosarcoma results from the administered
radiation being unable to destroy all viable cells, but is able
to induce malignant transformation.[9]
The clinical presentation of the parosteal variant of
osteosarcomas is as an expansile lesion on the involved bone,
with a nonlobular outer surface, and a potential for overlying
mucosal ulceration as was seen in our case.[1]
Radiographically, it is characteristically radiodense and
homogeneous, more at the base than at the periphery unlike
periosteal variety, which is not as radiodense and has a
poorly defined periphery. Parosteal osteosarcomas appear as
a lobulated nodule attached to cortical bone by means of a
short pedicle and usually overgrow its base of origin whereas
periosteal osteosarcoma tends to remain within the confines
of its base. There is no radiographic continuity with the
underlying marrow cavity in both varieties. The thin periosteal
radiolucency, about 1–3 mm in width (“string sign”) which
separates the tumor from the subadjacent cortex, is quite
characteristic of parosteal osteosarcoma, but is present only
in about 30% of the cases. New periosteal bone formation is
absent. Since treatment and prognosis varies with both the
variants of surface osteosarcomas, radiologic differentiation
is important.[3,5]
Due to the nonaggressive and noninvasive nature of parosteal
osteosarcoma, it must be distinguished from other benign
lesions such as osteoma or torus, osteoblastoma, and
osteochondroma; and several other differential diagnoses
can be considered as metastatic carcinoma, intramedullary
osteosarcoma, chondrosarcoma, and subpontic hyperostosis.[7,9]
Histologically, parosteal osteosarcoma is well‑differentiated,
characterized by spindle cell stroma with minimal atypia
Journal of Cancer Research and Therapeutics - Volume 14 - Issue 2 - January-March 2018
a b
Figure  6:  (a and b) Proliferation of well‑differentiated fibroblastic
cells with osteoid with absence of pleomorphism and atypical
mitosis (H and E, ×40)
and rare mitotic figures separating irregular trabeculae of
woven bone. It often presents with indolent behavior and a
bland‑looking microscopic appearance in the early stages of
the disease, and the final diagnosis is often only reached after
repeated biopsies or treatment as was seen in our case and
that reported by Ong et al.[10] These generally have a favorable
prognosis, better in mandible than maxilla when compared
to conventional and periosteal osteosarcomas. Treatment of
choice is wider resection with negative surgical margin.[1,5]
CONCLUSION
This case contributes to the literature of the rare variant
of osteosarcoma ‑ the parosteal osteosarcoma and must be
differentiated from other benign osseous lesions due to its
less aggressive biological behavior. The presence of soft tissue
mass arising from gingivobuccal sulcus in postradiation
cases must be viewed with a suspicion of juxtacortical
osteosarcomas.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
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 initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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