|Year : 2014 | Volume
| Issue : 1 | Page : 74-78
Osteosarcoma of mandible in a 10-year-old girl
SVSG Nirmala, Sivakumar Nuvvula, Kishore Kumar, Minor Babu, Sandeep Chilamakuri
Department of Paedodontics and Preventive Dentistry, Narayana Dental College and Hospital, Nellore, Andhra Pradesh, India
|Date of Web Publication||15-Feb-2014|
Department of Paedodontics and Preventive Dentistry, Narayana Dental College and Hospital, Nellore - 524 003, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Osteosarcoma (OS) of the jaws is a relatively rare malignant bone tumor. Like, its counterpart in the long bones, OS affecting the head and neck region shows distinct yet diverse clinical, histologic and prognostic characteristics. Here, we report a rare case of OS of fibroblastic variant in a 10-year-old girl, who came with a bony swelling in the left mandibular posterior region, with a radiographic presentation of sunburst appearance, the histopathological examination confirmed the diagnosis. The patient underwent partial mandibulectomy under general anesthesia followed by prosthodontic rehabilitation and is currently undergoing regular follow-up examination.
Keywords: Fibroblastic variant, mandible, osteosarcoma, partial mandibulectomy, sunray appearance
|How to cite this article:|
Nirmala S, Nuvvula S, Kumar K, Babu M, Chilamakuri S. Osteosarcoma of mandible in a 10-year-old girl. J Indian Soc Pedod Prev Dent 2014;32:74-8
|How to cite this URL:|
Nirmala S, Nuvvula S, Kumar K, Babu M, Chilamakuri S. Osteosarcoma of mandible in a 10-year-old girl. J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2019 Mar 22];32:74-8. Available from: http://www.jisppd.com/text.asp?2014/32/1/74/127067
| Introduction|| |
Osteosarcoma (OS) is a malignant mesenchymal tumor characterized by formation of osteoid tissue.  It is most common primary malignant bone tumor, accounting for approximately 20% of sarcomas but only 5% OS occur in jaws.  OS of jaw is uncommon and despite its histopathologic similarities with long bones, it is biologically different. 
OS is believed to arise from immature bone forming cells or through neoplastic differentiation of other immature mesenchymal cells into osteoblasts. Three main factors generally are proposed to be etiologically significant in their development - irradiation, pre-existing benign bone disorders and trauma  OS tumorigenesis has been linked to alterations in several genes. The first association of OS with an inherited predisposition was observed in patients with bilateral retinoblastoma. This association was confirmed by identification of the retinoblastoma susceptibility gene on human chromosome 13, which showed high percentage of mutations in OS.  The second gene associated with OS was p53 gene where the mutations in the p53 gene were first observed in sporadic OS. , In most cases, the neoplasm pursues an aggressive course.
Jaw OSs are usually present in the third and fourth decades of life, almost a decade after their presentation in long bone tumors with a slight predilection for the mandible. 
Here, we present a rare case report of OS in a 10-year-old girl. Clinical, radiographic, histopathologic features and management are discussed.
| Case Report|| |
The present case report is about a 10-year-old girl who presented with a 3 month history of painless swelling in the left back side of the lower jaw, which began growing rapidly and involving the cheek. The patient had no history of any trauma and not undergone treatment in this region. There was no history of any other disease affecting the jaw or other bones and medical, family history were noncontributory.
On extra oral examination [Figure 1] a well-defined swelling was detected unilaterally on the left side of the mandible extending anteroposteriorly from the left parasymphysis region to 2 cm anterior to the left angle of the mandible. Superior inferiorly, it extended from the level of the commissural region to the lower border of the mandible. The overlying skin was normal with a smooth surface. Upon palpation the swelling measured approximately 3 cm × 4 cm with no local rise in temperature, non-tender, firm in consistency toward the superior aspect and hard toward the inferior aspect. On intraoral examination [Figure 2] a swelling was observed in the left posterior buccal mucosa, measuring approximately 2 cm anteroposteriorly and from the occlusal plane to the depth of the buccal vestibular sulcus. The swelling was non tender; bidigital palpation revealed that the swelling in the buccal mucosa was firm, non-mobile and hard toward the depth of the sulcus. There was no evidence of mobility or decay in the vicinity of the swelling.
|Figure 2: An intraoral picture showing swelling in the left buccal mucosa|
Click here to view
Based on the sudden increase in size of the swelling, variable consistency of the nodule in the buccal mucosa and the patient's age, a provisional diagnosis of malignant tumor involving the body of the mandible and the adjacent soft tissue was made most likely Ewing's sarcoma. Differential diagnosis included OS, fibrosarcoma and chondrosarcoma.
Periapical radiograph [Figure 3] showed symmetrical widening of periodontal ligament space with altered bony trabecular pattern in relation to lower left second premolar and first permanent molar.
|Figure 3: Periapical radiograph showing symmetrical widening of periodontal space with altered trabecular pattern around second premolar and first permanent molar|
Click here to view
Orthopantomography [Figure 4] showed an increased radiolucency circumscribed by a well-defined radio-opaque rim extending from the distal margin of the crown of the second premolar, crossing the roots of the first permanent molar and extending inferiorly until the distal margin of the incompletely formed second molar.
|Figure 4: Panoramic radiograph showing a radiolucent lesion with patchy areas of radio opacity extending from distal aspect of first premolar to second molar with well-defined radio opaque rim|
Click here to view
Computed tomography (CT) [Figure 5] was performed to define precise extension of the lesion and revealed exophytic mass with a round radiolucent lesion measuring 4.2 cm × 3.1 cm with a partly obliterated rim involving the left lateral aspect of the posterior mandible as well as erosion of buccal cortical plate with characteristic "sun burst" appearance. This led to a radiological diagnosis of sarcoma, either OS or chondrosarcoma, involving the left lateral aspect of the mandible.
|Figure 5: Computed tomographic image showing erosion of buccal cortical plate with soft tissue involvement|
Click here to view
Histologically, OS is defined as a tumor characterized by the direct formation of osteoid or primitive woven bone by malignant tumor stroma cells.
Histopathological examination [Figure 6] of an incisional biopsy specimen revealed a sarcomatous stroma and tumor osteoid. Osteoid is recognized by eosinophilic staining quality, glossy appearance, irregular contours, surrounding rim of osteoblasts. Spindle shaped fibroblasts with hyperchromatic nuclei suggestive of fibroblastic variant of OS.
|Figure 6: Histopathological picture shows Spindle shaped fibroblasts cells with hyperchromatic nuclei and tumor osteoid|
Click here to view
Based on the radiographic and histologic findings, final diagnosis was made as a OS which was of fibroblastic variant. The treatment plan for this case was marginal mandibulectomy.
Under general anesthesia with endotracheal intubation, intra oral incision was made from left lower first premolar to retro molar area. Mucoperiosteal flap was reflected down to lower border of the mandible, lesion was identified and margin marked over the bone, marginal mandibulectomy was done from left lower first premolar to second permanent molar[Figure 7]. Hemostasis achieved, lesion closed with 3-0 vicryl suture. Recovery was uneventful [Figure 8].
During subsequent follow-up examination, a removable functional denture was inserted to prevent supra eruption of the maxillary left posterior teeth as well as to improve masticatory efficiency.
| Discussion|| |
World-wide, OS is considered to be a rare lesion occurring in about 1/100,000 persons/year; about 6-7% of all OSs occur in the maxillofacial region.  OS of the head and neck are considered by most clinicians to be distinct from OS that arise in the long bones. It is reported to affect older patients and follows a different clinical pattern.  In our case patient was very young. Jaw OSs usually present in third and fourth decades of life, almost a decade after their presentation in long bone tumors.  In the present case, in contrary to the above finding.
OS may arise de novo or subsequent to previous irradiation. It can also be seen in pre-existing Paget's disease of bone and Fibrous dysplasia. Isolated cases of trauma have been stated as contributing factors. The present case appears to have developed de novo; as no history of any predisposing factors to be elicited from this patient. Maxilla and mandible are equally involved. Mandibular tumors arise more frequently in posterior body and horizontal ramus.  In the present case, it arose in the posterior mandible which is compatible with earlier report.
The most common presenting symptom of OS in the head and neck region is swelling.  Our patient had swelling, this is in accordance with above finding. Pain has been reported in approximately 50% of patients in some large series,  although in other reports the occurrence of pain has been ranged from 3% to over 80% of patients.  It is interesting that patient in this report did not complain of pain. Mucosal ulceration and loosening of teeth can also occur. Pathological fractures may occur in cases of large sarcomas. Sensory neural abnormalities may occur in cases in which the lesion involves the course of peripheral nerves, involvement of temporomandibular joint or para mandibular musculature is often accompanied by trismus. , In contrary to above, our patient had no other symptoms.
Radiographically, OS usually produces poorly defined, irregular destructive bone lesion interspersed with radio opaque material. The indistinct margins corresponds to the infiltrative proliferation of malignant osteoblast and fibrous trauma, whereas the radio opaque foci represents the tumor bone/or cartilage produced in many lesions.  The lesion in the present case typifies this pattern. CT can be helpful in visualizing extramedullary involvement and extension into soft-tissues and is essential for early diagnosis of OS. 
OS involving the alveolar process may produce symmetrical widening of the periodontal ligament spaces as the neoplasm extends into the ligament.  This is in accordance with our finding. "Sunburst" pattern of tumor bone produced at right angles to the cortex is seen in no more than a third of all cases of OS.  Present case showed this pattern.
Histologically OS can be classified according to the cellular differentiation as osteoblastic, chondroblastic and fibroblastic. In osteoblastic type, atypical neoplastic osteoblasts exhibit considerable variation in shape and size, show large deeply staining, the chondroblastic type, consists of atypical chondroid areas composed of pleomorphic binucleate cells and fibroblastic type, shows atypical spindle shaped hyperchromatic cells.  The present case exhibits features of fibroblastic variant of OS.
Marx et al. reported that prognosis of a jaw OS is better than that of long bones OS. This could be due to histologically better differentiation of jaw OSs than long bone OS. As jaw OS occurs at higher mean age the patients have less chance of developing metastasis.  The prognosis of patients with OS has been reported to be statistically better with these factors : a0 bsence of parasthesia as a symptom, smaller tumor size, adequacy of surgical removal, a more differential histological grade of the lesion and younger age of the patient, increase age may be related to recurrence of the treatment.  Patients with mandibular lesions have been reported to have a longer median survival time than those with OS of the maxilla. 
| Conclusion|| |
OS of jaws presents to us with a wide spectrum of clinical, histologic and radiographic features, therefore all these features have to be correlated to reach a conclusive diagnosis. It may have a better prognosis, if diagnosed and treated at an early age.
| References|| |
|1.||Baghale K, Motahhary P. Osteosarcoma of the jaws: A retrospective study. Acta Med Iran 2003;4:113-21. |
|2.||Soares RC, Soares AF, Souza LB, Santos AL, Pinto LP. Osteosarcoma of mandible initially resembling lesion of dental periapex: A case report. Braz J Otorhinolaryngol 2005;71:242-5. |
|3.||Bennett JH, Thomas G, Evans AW, Speight PM. Osteosarcoma of the jaws: A 30-year retrospective review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:323-32. |
|4.||Garrington GE, Scofield HH, Cornyn J, Hooker SP. Osteosarcoma of the jaws. Analysis of 56 cases. Cancer 1967;20:377-91. |
|5.||Friend SH, Bernards R, Rogelj S, Weinberg RA, Rapaport JM, Albert DM, et al. A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature 1986;323:643-6. |
|6.||Iavarone A, Matthay KK, Steinkirchner TM, Israel MA. Germ-line and somatic p53 gene mutations in multifocal osteogenic sarcoma. Proc Natl Acad Sci U S A 1992;89:4207-9. |
|7.||Hansen MF. Genetic and molecular aspects of osteosarcoma. J Musculoskelet Neuronal Interact 2002;2:554-60. |
|8.||Ogunlewe MO, Ajayi OF, Adeyemo WL, Ladeinde AL, James O. Osteogenic sarcoma of the jaw bones: A single institution experience over a 21-year period. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:76-81. |
|9.||Balwani SR, Tupaki JV, Barpande SR. Parosteal osteosarcoma of the mandible. J Oral Maxillofac Pathol 2006;10:10-4. |
|10.||Nissanka EH, Amaratunge EA, Tilakaratne WM. Clinicopathological analysis of osteosarcoma of jaw bones. Oral Dis 2007;13:82-7. |
|11.||Mark RJ, Sercarz JA, Tran L, Dodd LG, Selch M, Calcaterra TC. Osteogenic sarcoma of the head and neck. The UCLA experience. Arch Otolaryngol Head Neck Surg 1991;117:761-6. |
|12.||Okinaka Y, Takahashi M. Osteosarcoma of the maxilla: Report of a case and review of the literature concerning metastasis. J Oral Maxillofac Surg 1997;55:1177-81. |
|13.||Tanzawa H, Uchiyama S, Sato K. Statistical observation of osteosarcoma of the maxillofacial region in Japan. Analysis of 114 Japanese cases reported between 1930 and 1989. Oral Surg Oral Med Oral Pathol 1991;72:444-8. |
|14.||Adekeye EO, Chau KK, Edwards MB, Williams HK. Osteosarcoma of the jaws - A series from Kaduna, Nigeria. Int J Oral Maxillofac Surg 1987;16:205-13. |
|15.||Bianchi SD, Boccardi A. Radiological aspects of osteosarcoma of the jaws. Dentomaxillofac Radiol 1999;28:42-7. |
|16.||Givol N, Buchner A, Taicher S, Kaffe I. Radiological features of osteogenic sarcoma of the jaws. A comparative study of different radiographic modalities. Dentomaxillofac Radiol 1998;27:313-20. |
|17.||Lee YY, Van Tassel P, Nauert C, Raymond AK, Edeiken J. Craniofacial osteosarcomas: Plain film, CT, and MR findings in 46 cases. AJR Am J Roentgenol 1988;150:1397-402. |
|18.||Cavalcanti MG, Ruprecht A, Yang J. Radiological findings in an unusual osteosarcoma in the maxilla. Dentomaxillofac Radiol 2000;29:180-4. |
|19.||Marx RE, Stern D. Oral and Maxillofacial Pathology a Rationale for Diagnosis and Treatment. Carol Stream: Quintessence Publishing Co. Inc.; 2003. |
|20.||Daw NC, Mahmoud HH, Meyer WH, Jenkins JJ, Kaste SC, Poquette CA, et al. Bone sarcomas of the head and neck in children: The St Jude Children's Research Hospital experience. Cancer 2000;88:2172-80. |
|21.||Patel SG, Meyers P, Huvos AG, Wolden S, Singh B, Shaha AR, et al. Improved outcomes in patients with osteogenic sarcoma of the head and neck. Cancer 2002;95:1495-503. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]