|Year : 2008 | Volume
| Issue : 1 | Page : 41-44
Osteosarcoma of jaw bone
Avani Gandhi Dixit, Nipa J Parikh
Department of Oral Medicine and Radiology, Dr. D. Y. Patil Dental College and Hospital, Pimpri, Pune-411 018, Maharashtra, India
Avani Gandhi Dixit
Department of Oral Medicine and Radiology, Dr. D. Y. Patil Dental College and Hospital, Pimpri, Pune. 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Osteosarcoma is the most common primary malignant bone tumor in children. Its radiolographic appearances vary considerably. It may show a number of presentations from onion skin or sunburst appearance. Osteosarcoma of maxilla and mandible are distinct from long bone osteosarcoma and shows a distinct clinical, histologic and prognostic characteristic that when diagnosed and treated properly have a better prognosis from the later. The present article reports a case of osteogenic sarcoma of maxillary alveolar ridge and compares it with long bones osteosarcomas.
Keywords: Osteosarcoma, sunburst appearence
|How to cite this article:|
Dixit AG, Parikh NJ. Osteosarcoma of jaw bone. J Indian Acad Oral Med Radiol 2008;20:41-4
| Introduction|| |
Osteosarcoma is the second most common primary malignant tumor of bone. Osteosarcoma of head and neck are rare tumor and constitute less than 10% of all osteosarcomas. ,,, They occur with equal frequency in maxilla and mandible. , Lesions are slightly more common in men and usually occur in the third and fourth decade of life. ,,, Osteosarcoma of long bones is more common in children. Primary osteosarcoma of jaw bones are a group of lesions which are biologically distinct from long bones osteosarcoma and have a slightly better prognosis than them.
| Case Report|| |
A 42 year old female patient reported to Dr. D.Y. Patil Dental College and Hospital, Pimpri, Pune, with a chief complaint of pain and swelling in upper left posterior quadrant of jaw since past three months [Figure 1]. The swelling was gradually increasing in size. The patient gave a history of exfoliation of a molar tooth from the same region 2 years back.
On clinical examination, there was a diffuse swelling seen on the left side of the face, extending from the bridge of the nose to the outer canthus of the eye; and vertically extending from the lower border of orbit to the upper border of the lips. The skin over the swelling was tensed, with no ulcerations and was warm to touch.
Intraoral examination revealed a well defined swelling in the maxillary arch, extending from the first premolar back up to the distal of second molar on left side. The lesion appeared reddish pink in color, with the overlying surface normal. On palpation, the lesion was firm in consistency, compressible as well as fluctuant. The teeth in association with the lesion i.e. 24 and 27 exhibited grade III mobility.
Intra oral periapical of the region revealed a non corticated, well defined, mixed radiolucent-radiopaque lesion denoting irregular areas of osteolysis and spotty ossifying new bone formation overgrowing the alveolar crest [Figure 2]. The patient's panoramic radiograph also revealed similar mixed radiolucent-radiopaque lesion. The occlusal view taken showed similar lesion with streaking at the periphery, suggestive of osteoblastic activity and new bone formation. The posterio-anterio Caldwell view revealed diffuse radiopacity involving the left maxillary sinus completely.
Given the patient's clinical and radiological findings, a provisional diagnosis of osteogenic sarcoma was given.
A decision was made to surgically excise the tumor completely along with a margin of normal tissue and then give the patient an immediate obturator for the defect eventually produced. Accordingly, the impressions were made before the surgery and the outline of the defect marked subjectively; the obturator was made before hand and then adjusted in the patient's mouth immediately after the resection of the tumor.
Eventually, the recected specimen was sent for histopathologic evaluation. Lesional tissue showed many proliferating, atypical and neoplastic osteoblasts. Intermittent tumor osteoid was also seen in most areas. Areas of atypical chondroid tissue were seen with large chondrocytes. Areas of focal calcification were also evident. The lesion appeared well circumscribed in one section with intact overlying oral epithelium. The picture confirmed the report of osteogenic sarcoma [Figure 3].
| Discussion|| |
Osteosarcoma of jaws is uncommon and constitutes less than 10% of all osteosarcomas and 15% of all primary bone tumors confirmed at biopsy. ,,, Despite its histopathological similarity with long bones osteosarcoma, it is biologically different from them.  The median age of occurrence is one or two decade higher than patients with long bones osteosarcoma,  most of the patients are in third and fourth decades of life. ,,, Lesions are slightly more common in men. ,, They occur with almost equal frequency in both the jaws. , The most common places of occurrence are the alveolar ridge and the body of maxilla and mandible. ,,,,, The median age of maxillary osteosarcoma is reported to be higher than the mandibular one. The main symptom of this lesion in jaw is swelling followed by pain.  Although the pathogenesis is unknown, various predisposing factors have been proposed. These include preexisting bone lesions like bone cysts, osteogenesis imperfecta, osteochondroma, fibrous dysplasia, trauma, genetic factors, virus and previous radiation.
Depending upon the characteristic, osteosarcoma can be differentiated into primary; that may be central/intramedullary or surface/extraskeletal, and secondary to malignant transformation of benign tumors such as Paget's disease, fibrous dysplasia, or other causes such as radiation exposure, chemotherapy or foreign bodies.
The World Health Organization's histologic classification of bone tumors separates the osteosarcomas into central (medullary) and surface (peripheral) tumors and recognizes a number of subtypes within each group [Table 1]. The most common pathologic subtype is conventional high-grade central osteosarcoma. It accounts for 80-90% of all osteosarcomas and is characterized by areas of necrosis, atypical mitoses and malignant cartilage. Its most frequent subtypes are osteoblastic, chondroblastic and fibroblastic osteosarcomas.
The extent of the tumor in both bone and soft tissue is best appreciated with cross sectional imaging techniques such as computerized tomography (CT) or magnetic resonance imaging (MRI) [Figure 4]. This is particularly important prior to definitive surgery. 
A radionuclide bone scan, with methylene diphosphonate labeled technetium 99m defines the extent of the primary tumor and as its uptake will extend slightly beyond the limits of tumor, it helps to define a safe margin in surgical planning. It is also helpful in the detection of "skip lesions" within the same bone, as well as distant bone metastases.
Because of the frequent presence of pulmonary metastasis at diagnosis (15-20%), CT of the chest is essential; these lesions often appear as calcified nodules.
Micrometastatic disease is present at diagnosis in 80-90% of patients but undetectable with any of present tests.
There are no known specific laboratory parameters. Increases of alkaline phosphatase or lactic dehydrogenase (LDH) serum level are observed in a considerable number of patients. Although they do not correlate reliably with disease extent, they may have negative prognostic significance.
The diagnosis of osteosarcoma must be verified histologically with a biopsy before initiation of treatment [Figure 5]. A core needle biopsy under local or general anaesthesia is the most widely used technique. However, open biopsy may be performed to obtain sufficient material for histological evaluation and ancillary studies.
Histologically, jaw osteosarcomas have better differentiation than long bone osteosarcoma.  The diagnosis of these lesions is based on osteoid production by malignant cells, although these cells are also able to produce chondroblastic or fibroblastic extracellular matrix. Designation as osteoblastic or chondroblastic variety is of clinical significance as it has been reported that the later has a marginally better prognosis. Radiographically, a combination of sunray appearance, Periodontil ligament widening and widening of mandibular canal are pathognomonic for jaw osteosarcoma. ,
Grading and staging
Cellularity is the most important criterion used for histological grading. In general, the more cellular the tumor, the higher the grade is. Irregularity of the nuclear contours, enlargement and hyperchromasia of the nuclei are correlated with grade. Mitotic features and necrosis are additional features useful in grading
Staging incorporates the degree of differentiation as well as local and distant spread, to estimate the prognosis of the patient. The universal TNM staging system is not commonly used for sarcomas because of their rarity to metastasize in lymph nodes. The system used most often to formally stage bone sarcomas is known as the Enneking system. It is based on the grade (G) of the tumor, the local extent of the primary tumor (T), and whether or not it has metastasized to regional lymph nodes or other organs (M).
The grade is divided into low grade (G1) and high grade (G2).
The extent of the primary tumor is classified as either intracompartmental (T1), meaning it has basically remained in place, or extracompartmental (T2), meaning it has extended into other nearby structures.
Tumors that have not spread to the lymph nodes or other organs are considered M0, while those that have spread are M1.
These factors are combined to give an overall stage.[Additional file 1]
In summary, low-grade tumors are stage I, high-grade tumors are stage II, and metastatic tumors (regardless of grade) are stage III.
Osteosarcoma can be localized or metastatic. Localized tumors are limited to the bone of origin, although local skip metastases may be apparent within the bone, indicating a worse prognosis.
It is believed that jaw osteosarcomas behave differently from long bone osteosarcomas. There are several ways in which this difference becomes apparent:
- Jaw osteosarcomas exhibit a mean age of occurrence one decade higher than long bones osteosarcomas.
- The metastatic rate of jaw osteosarcomas range from 6 to 51% whereas that of long bones varies from 78 to 90%.
- The prognosis of jaw osteosarcomas is better than long bone osteosarcomas.
Treatment of this lesion is radical surgery consisting of complete resection along with a margin of normal surrounding tissue,  usually accompanied by chemotherapy.  Anatomical limitations in face can cause some difficulties in achievement of uninvolved margins and for this reason local recurrence of these lesions is high. , The rate of metastasis in this lesion is less than long bones osteosarcoma (33-69%). , Diagnosis of tumors in early stages and complete resection are most important factors in increasing the prognosis of jaw osteosarcoma. 
| References|| |
|1.||Clark JL, Unni KK, Dahlin DC, Devine KD. Osteosarcoma of the jaw. Cancer 1983;51:2311-6. [PUBMED] |
|2.||Smith AC, Harvey L. Chondroid osteosarcoma of the Maxilla. Int J Oral Maxillofac Surg 1987;16:232-5. [PUBMED] |
|3.||August M, Magennis P, Dewitt D. Osteogenic sarcoma of the jaws: Factors influencing prognosis. Int J Oral Maxillofac Surg 1997;26:198-204. [PUBMED] |
|4.||Vege DS, Borges AM, Aggrawal K, Balasubramaniam G, Parikh DM. Bhaser B. Osteosarcoma of the craniofacial bones: A clinico-pathological study. J Craniomaxillofac Surg 1991;19:90-3. |
|5.||Tanazawa H, Uchiyama S, Sato K. Statistical observation of osteosarcoma of the maxillofacial region in Japan. Oral Surg Oral Med Oral Pathol 1991;72:444-8. |
|6.||Bennett JH, Thomas G, Evans AW, Speight PM. Osteosarcoma of the jaw: A 30 years retrospective review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:323-33. [PUBMED] [FULLTEXT]|
|7.||Bertoni F, Dallera P, Bacchini P, Marchetti C, Campobassi A. The institute Rizzoli-Beretta experience with osteosarcoma of the jaw. Cancer 1991;68:1555-63. [PUBMED] |
|8.||Mardinger O, Givol N, Talmi YP, Taicher S. Osteosarcoma of the jaw. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:445-51. [PUBMED] [FULLTEXT]|
|9.||Schajowicz F, Sissons HA, Sobin LH. The World Health organization's Histologic classification of bone tumors: A commentary on the second edition. Cancer 1995;75:1208-14. [PUBMED] |
|10.||Slootweg PJ, Muller M. Osteosarcoma of the jaw bones. J Maxillofac Surg 1985;13:158-66. |
|11.||Forteza G, Colmenero B, Lopez-Barea F. Osteogenic sarcoma of the maxilla and mandible. Oral Surg Oral Med Oral Pathol 1986;62:179-84. |
|12.||Kumar R, Moser RP, Madewell JE, Edeiken J. Paraosteal osteogenic sarcoma arising in cranial bones: Clinical and radiological features in eight patients. AJR Am J Roentgenol 1990;155:113-7. |
|13.||Lewis M, Perl A, Som PM, Urken ML, Brandwein MS. Osteogenic sarcoma of the jaw: A clinicopathologic review of 12 Patients. Arch Otolaryngol Head and Neck Surg 1997;123:169-74. |
|14.||Lindquist C, Teppo L, Sane J, Holmstrom T, Wolf J. Osteosarcoma of the mandible: Analysis of nine cases. J Oral Maxillofac Surg 1986;44:759-64. |
|15.||Soderholm AL, Lindquist C, Teppo L, Wolf J, Sane J. Bone resection in patients with mandibular sarcoma. J Craniomaxillofac Surg 1988;16:224-9. |
|16.||Rosen G, Caparros B, Huvos AG, Kosloff C, Nirenberg A, Cacavio A, et al . Preoperative chemotherapy for Osteogenic sarcoma: Selection of postoperative Adjuvant chemotherapy based on the response of the primary tumor to preoperative chemotherapy. Cancer 1982;49:1221-30. [PUBMED] |
|17.||Kawasaki T, Ono N, Watanabe K, Koshi K. Chondroblastic osteosarcoma of the mandible: Report of a case. J Oral Maxillofac Surg 1996;54:1123-7. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]