Osteosarcomas of the jaws are challenging lesions in many ways. These tumors are very deforming: they occur in the anatomical region that defines an individual the most, and therefore any deformity here affects the individual in many areas of life. Nothing is more psychologically depressing than rejection by one’s own family, as was the status of the patient presented here. Surgical resection of these tumors is another challenge: adequate resection of these tumors is difficult, especially of the maxilla, because of the complexity of the anatomy, and the resultant defect. Defect reconstruction is a further challenge: facial height and width must be maintained or reconstructed. While the potential for complications in the resection of both the mandible and maxilla is significant, the potential gain even for a purely palliative excision is great, given the cruelty of death from starvation and suffocation that the patient would otherwise have had to face.
The difficulties with histological and radiological diagnosis of osteosarcomas of the jaw are well documented [3, 4, 16, 19–21]. In the case presented here, a provisional diagnosis of juvenile ossifying fibroma was initially entertained, with international consultations leading to a consensus diagnosis of osteosarcoma of both the mandible and the maxilla.
The ‘sunburst’ appearance, the classic radiologic appearance of long-bone osteosarcomas, is not pathognomonic for jaw osteosarcomas. Indeed, the radiologic appearance of jaw osteosarcomas depends on the interplay of three processes: bone formation and mineralization, bone destruction, and periosteal bone formation. Radiologically, the lesions may range from predominantly radiolucent to radio-opaque lesions, with any combinations thereof, depending on the degree of ossification . Plain radiographs and CT scans may be used to define the tumor extent and may thus aid in deciding on resectability, while a chest radiograph or CT scan may be used to detect lung metastasis.
While the initial aim of the surgery was palliative debulking, the resection margins obtained gave hope for the possibility of a cure, especially if adjuvant radiotherapy could be accessed in good time, before any recurrence.
Some authors have suggested that tumor size, location, and histologic grade are the main prognostic factors, although this has been disputed , with others stating that tumor size does not appear to be of prognostic value . Early diagnosis and adequate surgical resection are essential for survival, with control of local spread assuring short-term survival. Where adequate resection is possible, surgery alone is adequate, while adjuvant therapy should be considered where surgical margins are inadequate [3, 9]. Some authors have suggested that craniofacial osteosarcomas are high-grade tumors, which would suggest a poor outcome in general, but this is not borne out in the general literature, and may be rather an expression of a population-specific variable [7, 16]. Only 9% of craniofacial osteosarcomas in a study by Jasnau et al.  were high-grade tumors.
Daffner et al.  reported an unusually aggressive osteosarcoma of the mandible, with widespread systemic metastases and death within four months of diagnosis. In contrast, many authors have found most jaw osteosarcomas to be well differentiated [23, 25].
Because of easier resectability, and the ability to obtain negative surgical margins, mandibular osteosarcomas have a better prognosis than maxillary tumors . Granowski-LeCornu et al. confirmed a poorer outcome for maxillary tumors, related to the difficulty in obtaining adequate resection margins, leading to increased incidence of local recurrence, residual disease, and death. Long-term survival is dependent on such variables as local recurrence, intracranial invasion, and distant spread.
Thus, wide surgical resection forms the most important intervention in the management of osteosarcomas of the jaws, with chemotherapy and irradiation being adjuvant modes, or primary modes in instances of palliation for unresectable lesions .
While adjuvant chemotherapy may improve early survival, neither chemotherapy nor radiotherapy appears to impact long-term survival. Because positive surgical margins are associated with poor prognosis, some authors have shown that the use of multimodal therapy may significantly reduce rates of local recurrence, and thereby improve survival [14, 18, 19, 27].
The likelihood of cure in osteosarcomas of the jaws has been estimated at 60% to 70% . Nevertheless, palliative resection, when feasible, even for extensive tumors such as the one reported by the current author, offers the potential for an immediate improvement in the quality of life, including the ability to re-integrate with society, feed, and regain speech and a highly increased degree of self-worth and acceptance.
Multicentric osteosarcomas, defined as lesions present in more than one bone, typically in the absence of pulmonary or other visceral metastases, represent 1 to 2% of all osteosarcomas. They may be synchronous (multiple tumor foci within 5 months of initial presentation) or metachronous (multiple tumor foci 5 months after initial presentation) [24,28,]. Multicentric osteosarcoma cell lines have increased invasiveness and metastatic ability, as well as an affinity for bone; additionally, they have a uniformly poor response to multimodal therapy. Synchronous osteosarcomas (dominant lesions found with smaller synchronous lesions), are associated with a poorer prognosis, because of rapid disease progression and death . The debate as to whether multicentric osteosarcomas represent primarily metastatic disease or multiple primaries is unsettled [28, 29]. Another entity found in up to 10% of osteosarcomas is ‘skip’ metastases, where a second focus of osteosarcoma is found in the same bone or in an adjacent bone across a joint [29, 30].
It is instructive that, with the exception of a few reports [4, 24], almost all the conclusions on the behavior of multicentric osteosarcomas are based on long-bone tumors, which are known to differ significantly in their biological behavior from that of tumors of the jaws, and cannot, therefore, be representative of multicentric osteosarcomas of the jaws [28–30]. Indeed, to date, there are only four reports of multicentric osteosarcomas involving the jawbones in the English literature [31–34].
Behere and Lele  reported on a 16-year-old girl found to have a mandibular osteosarcoma and multiple synchronous skeletal lesions. Stroncek et al.  similarly reported on a 15-year-old girl with mandibular and ilium osteosarcomas, and postmortem pulmonary metastases. They hypothesized that the mandibular tumor was metastatic from the ilium. Ohba et al.  reported on a 17-year-old girl who presented with mandibular metastasis from an osteosarcoma of the fibula; she subsequently developed systemic metastases. Zhang et al.  reported on an 18-year-old girl with synchronous osteosarcoma lesions in the mandible and maxilla. Of all the cases of multicentric jaws osteosarcomas in English literature to date, three represent obvious systemic metastatic disease [31–33], with only one  involving only the jaws. Interestingly, all the four cases reported involved teenage girls [31–34].
Thus, the current case report represents the first male patient with synchronous jaw osteosarcomas, and the only one from sub-Saharan Africa. The absence of other lesions and the clinical and radiological presentation in the current case lends credence for the synchronous multicentric theory in the evolution of these osteosarcomas. These lesions are most likely to have developed independently in two different anatomical sites, in the same individual. Their size, and the time to surgical excision, is evidence of the different biological behavior exhibited by these tumors, and the better prognosis associated with jaw osteosarcomas when compared with long-bone osteosarcomas. Thus the report by Zhang et al.  and this report serve as evidence that multicentric jaw osteosarcomas do not necessarily always represent a continuum of metastatic osteosarcoma disease, as proposed by some authors [24, 28]. Indeed, one may conclude that the older three reports on multicentric jaw tumors represent purely metastatic disease, from long bones in a disease known to be aggressive, and should not, therefore, be used as evidence of the process of evolution of jaw osteosarcomas [31–33].
Evidence from most studies has shown that jaw osteosarcomas rarely metastasize, lending further weight to the argument that apparently multicentric osteosarcomas involving the jaws and the appendicular skeleton are more likely to be metastatic from the long bones [24, 28, 31–33].
All authors appreciate the different behavioral patterns of gnathic osteosarcomas compared with long-bone osteosarcomas. It is, therefore, entirely possible that well-differentiated osteosarcomas represent an entirely different entity, one that is related to but is not an actual osteosarcoma. Such an entity would explain the difficulties abounding in the literature on histological and radiological diagnosis, differences in response to treatment, and rarity of metastatic disease with, therefore, a more favorable prognosis of gnathic osteosarcomas . Perhaps the time has come for histopathologists to re-examine diagnostic criteria for jaw osteosarcomas.