|Year : 2021 | Volume
| Issue : 2 | Page : 379-381
Periosteal osteosarcoma of the mandible: A diagnostic challenge
Suvarna Deshpande, Rajasbala Dhande, Shirish Vaidya, Kaustubh Madurwar
Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, Maharashtra, w, Wardha
|Date of Submission||10-Jun-2020|
|Date of Decision||22-Oct-2020|
|Date of Acceptance||12-Jan-2021|
|Date of Web Publication||18-Oct-2021|
Dr. Suvarna Deshpande
Department of Radiodiagnosis, Datta Meghe Institute of Medical Science, Sawangi, Wardha, Maharashtra
Source of Support: None, Conflict of Interest: None
This is a case report of a rare malignant mesenchymal tumor, juxtacortical (periosteal) osteosarcoma arising from the mandible in a 17 year old boy. About 4%-6% of osteosarcomas occur in the maxillofacial region, while incidence of osteosarcoma occurring in jaw is 0.7 per million population. Due to its unique feature , they are high number of chances of misdiagnosing the tumor with other peripheral fibro-osseous lesions. The prognosis of the tumor is good if detected early and a wide margin resection is performed to make the recurrence obsolete.
Keywords: Malignant bone tumour, juxtacortical osteosarcomas, Mandible and maxilla
|How to cite this article:|
Deshpande S, Dhande R, Vaidya S, Madurwar K. Periosteal osteosarcoma of the mandible: A diagnostic challenge. J Datta Meghe Inst Med Sci Univ 2021;16:379-81
|How to cite this URL:|
Deshpande S, Dhande R, Vaidya S, Madurwar K. Periosteal osteosarcoma of the mandible: A diagnostic challenge. J Datta Meghe Inst Med Sci Univ [serial online] 2021 [cited 2021 Dec 9];16:379-81. Available from: http://www.journaldmims.com/text.asp?2021/16/2/379/328454
| Introduction|| |
A malignant bone tumor, juxtacortical osteosarcoma appearing in the mandible or maxilla, is uncommon, which accounts 7% of all osteosarcoma and 1% of all the head and neck malignancy. Mandible and maxilla are equally involved. It has more male predilection than females. The average onset age of osteosarcoma of the jaw is 10–20 years. The survival rates of these unconventional type of osteosarcomas are higher as there is a difference between the biologic behavior of osteosarcoma involving other skeletal bones and those arising from the jaw. In general, osteosarcomas arising from the mandible and maxilla are highgrade lesions. Lowgrade lesions are uncommon, which includes welldifferentiated intramedullary osteosarcoma (lowgrade central osteosarcoma) and periosteal osteosarcoma.
The predisposition for osteosarcoma in young patients is due to somatic or germline mutations. The cause of osteosarcoma is mainly due to Rb and p53 inactivation which are tumor suppressor genes present on 17p13 and 13q14, respectively. Mutation in these genes causes uncontrolled proliferation and malignant transformation.
Juxtacortical osteosarcoma has further two types, i.e., parosteal osteosarcoma and periosteal osteosarcoma. Because of their rarity, they are frequently misdiagnosed as other lesions such as myositis ossificans, osteochondromas, and chondrosarcomas, leading to inadequate marginal resection.
| Case Report|| |
A 17-year-old boy presented with swelling over the right lower jaw for 1 month, which was sudden in onset, rapid in progression, continuous in growth, and associated with dull aching pain. There was a history of difficulty in mastication, deglutition for 8 days. There was a history of appetite loss for 8 days and weight loss (4 kg) for 15 days. On extraoral examination, face was grossly asymmetrical due to swelling from the angle of the mouth to the tragus on the right side of the lower jaw. The swelling is firm in consistency with tenderness and induration present over it. Palpable cervical lymphadenopathy was present. There was no bleed and pus discharge [Figure 1]. Hard palate, soft palate, tongue, and floor of the mouth appeared normal. Blood investigation with hemoglobin: 12.5 g%, total lung capacity: 8000/mm3, platelet count: 2.2 lac/mm3, red blood cell: 4.4 million/mm3, and kidney function test: normal, but liver function test revealed 154 mg/dl (raised alkaline phosphatase level). A detailed radiological evaluation was performed. On ultrasound, there was heterogeneously hypoechoic mass lesion with a few cystic areas within adjacent to the right lower jaw extending from the angle of the mouth to the tragus, with associated submandibular lymphadenopathy. It showed hyperechoic linear strands within arising from the mandibular cortex radially. On computed tomography (CT), there was a heterogeneously enhancing multilobulated mass lesion with extra- and intraoral extension on the right side, causing erosion of the adjacent mandible [Figure 2]a. There were hyperdense strands arising from the mandibular cortex from both sides radially outward, giving a typical sunburst appearance [Figure 2]b. The lesion is situated predominantly in the right masticator space involving the sublingual space and submandibular space [Figure 3]a. On axial CT bone window, there was an incomplete triangular hyperdensity noted arising from the body of the mandible, which is aggressive disorganized type of periosteal reaction perpendicular to the cortex of the mandible. There was associated cortical thinning and erosion of the adjacent bone with destruction of the interalveolar septas [Figure 3]b. There is e/o hyperdense strands noted arising from the mandibular cortex from both sides radially outward giving a typical sunburst appearance. The biopsy results showed that it was a juxtacortical periosteal type of osteosarcoma, chondroblastic variant.
|Figure 1: On ultrasound, there was heterogeneously hypoechoic mass lesion with a few cystic areas within adjacent to the right lower jaw extending from the angle of the mouth to the tragus, with associated submandibular lymphadenopathy. It showed hyperechoic linear strands within arising from the mandibular cortex radially|
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|Figure 2: (a) An ill-defined heterogeneously enhancing lesion with extra- and intraoral extension on the right side causing erosion of the adjacent mandible. There is e/o hyperdense strands noted arising from the mandibular cortex from both sides radially outwards giving a typical sunburst appearance. (b) The lesion is situated predominantly in the right masticator space involving the sublingual space and submandibular space|
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|Figure 3: (a) On axial computed tomography bone window, there is an incomplete triangular hyperdenisty noted arising from the body of the mandible, which is aggressive periosteal reaction perpendicular to the cortex of the mandible forming a cod man triangle. There is associated cortical thinning and erosion of the adjacent bone with destruction of the inter alveolar septas. (b) There is e/o hyperdense strands noted arising from the mandibular cortex from both sides radially outwards giving a typical sunburst appearance|
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| Discussion|| |
Craniofacial osteosarcomas are situated in the mandible or maxilla and have attributed 6%–13% of all osteosarcoma juxtacortical (periosteal) osteosarcomas are extraskeletal, mostly soft tissue lesions, a nonconventional type of osteosarcoma and it has a better prognosis than central lesions.
On histology, there are variants of osteosarcoma which are of osteoblastic, chondroblastic, and fibroblastic types. A very few are telangiectatic, small cell, malignant fibrous histiocytoma type of osteosarcomas. It is due to direct bone or osteoid formation by the malignant neoplastic cell. The proportion of osteoid and ossified tissue varies in various types of tumors. Osteoid can again be well-defined structures that mimic trabecular bone or be irregular deposits.,
Radiographically, juxtacortical (periosteal) osteosarcomas are radiodense, lobulated, cauliflower-like, or oval masses. The stalk is broad and has an attachment to the external cortex of the underlying bone. Radiodensity increases from the periphery to the base. Periosteal variant is considered to arise from the outer layer of the cortex. Other radiographic patterns which prompt toward the diagnosis of juxtacortical osteosarcoma like cotton wool image with distinct margin, patchy radiopacity and tumor mass with diffuse hazy opacification with few fine bony spicules radiating from the lower border of the lesion. Periosteal osteosarcoma consists of the irregular trabeculae which are separated by a spindle cell stroma with minimal atypia and mitotic figures. Osteochondroma, which area rarely found in jaw, shows continuity of cortex and underlying medullary bone with the base of lesion. Periosteal osteosarcoma has an overall good prognosis and has low metastatic potential. Parosteal and periosteal types tend to have a better prognosis than conventional osteosarcoma or high-grade surface osteosarcoma in this case the person was treated with segmental mandibulectomy, and followed up. Follow-up is very important to keep recurrence in check.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]