In the head and neck region, osteosarcoma is the most common primary malignant bone tumor, representing 23% of total head and neck malignancies. Osteosarcomas of the jaws are nevertheless rare lesions, representing only 2 to 10% of all osteosarcomas. This report reviews a single-center histopathology experience with craniofacial osteosarcomas, and reports the management of unusually large synchronous mandibular and maxillary osteosarcomas in a patient.
Patients and methods
A search of the hospital pathology database for specimens with a histological diagnosis of osteosarcomas submitted between July 1992 and May 2011 was made. A chart review of a patient with large synchronous maxillary and mandibular osteosarcomas was performed, and is reported.
A 21-year-old African man with large maxillary and mandibular tumors under palliative care presented with increasing difficulties with eating, speech, and breathing. Surgical debulking was performed, with histology confirming synchronous osteosarcomas of the mandible and maxilla. The patient is well after one year, with no evidence of recurrence, having undergone no further treatment.
Osteosarcomas of the jaw remain enigmatic, and a number of difficulties related to their diagnosis and treatment are yet to be resolved. True synchronous multicentric osteosarcomas of the jaws are extremely rare but, like other osteosarcomas of the jaws, have a favorable outcome, and palliative resection of such lesions, though challenging, can therefore lead to an enormously improved quality of life and self-image, and may even offer the opportunity for cure.
Keywords: Jaw osteosarcomas; Multicentric; Palliative surgery; Synchronous
Osteosarcoma is the most common primary malignant bone tumor, and in the jaws represents up to 23% of total head and neck malignancies [1,2]. Osteosarcomas of the jaws are, however, rare lesions, representing only 2 to 10% of all osteosarcomas [3-5]. Ten percent of these lesions are radiation induced. Hereditary retinoblastoma, Paget’s disease of bone, a history of fibrous dysplasia, or trauma are other factors known to predispose to the development of osteosarcomas [6-9].
Reports on osteosarcoma of the jaws from Africa are few; most reports originate from Nigeria, Kenya, and South Africa [2,3,8,10-12]. Adekeye et al.  reported finding demographic characteristics in their patient population that were similar to those reported in Western literature. Chindia et al.  reported on 14 cases of jaw osteosarcomas in patients with a mean age of 30 years (one week to 50 years). The mandible was the most commonly involved bone, as in other studies [1,2,11]. Ogunlewe et al.  reported that osteosarcoma of the jaw constituted 0.6% of lesions of the jaws and oral cavity over a 21-year period in a Nigerian institution; the patients’ mean age was 27 years. While mandibular lesions occurred predominantly in women, their study suggested a male predisposition of maxillary lesions .
Patients with osteosarcomas of the jaw are generally 10 to 20 years older, on average, than those with osteosarcomas of long bones . They have a male to female ratio of 1:1. Studies differ as to the most commonly involved bone [1,2,5,14].
As with a number of other pathologies in sub-Saharan Africa, late presentation of patients with osteosarcoma of the jaws is a common feature [3,8,11]. Thus, the use of adjuvant chemo-radiotherapy with surgical extirpation does not appear to affect outcomes, primarily as the result of late presentation [3,8].
The primary presenting complaints are pain, swelling, paresthesia, and ulceration . Osteosarcomas of the jaws have a different biological behavior from that of osteosarcomas of the long bones, with a lower incidence of metastases and a much better prognosis. This conclusion is based on findings from many case reports and small case series [4,15,16]. Studies on larger cohorts of patients with osteosarcomas of the jaw have suggested a prognosis equivalent to that of long-bone osteosarcomas, which is therefore poorer than previously thought; a conclusion that is not widely accepted at the present time [17,18].
Unusual presentations of osteosarcomas of the jaws, the continuing challenges in establishing both radiological and histological diagnosis, and controversies in treatment and prognosis are the reasons for presenting this case report and literature review.
Patients and methods
A search of the hospital pathology database for specimens with a histological diagnosis of osteosarcomas submitted between July 1992 and May 2011 was made. A chart review of a patient with large maxillary and mandibular tumors found to be osteosarcomas was drawn up, and the results presented. This study did not require the approval of the hospital ethics committee because it is a retrospective or chart review study.
A total of 235 specimens submitted between July 1992 and May 2011 to the AIC Kijabe Hospital pathology department were reported histologically as osteosarcomas. Of six patients with osteosarcomas of the craniofacial region, five had jaw tumors. There were two men and three women, with a mean age of 29.5 years. Jaw osteosarcomas represented 2.1% of all osteosarcomas recorded during the study period.
A 21-year-old African man presented with a two-year history of facial tumors. He had no other significant medical or family history. He had initially noted a small swelling over his right maxilla, and subsequently felt another in his mandible. The two masses grew over time, even as he sought medical attention. The prohibitive cost of the surgery prevented him from accessing surgical care. At the end of two years, and now with the maxillary and mandibular tumors having grown so large that they interfered with his eating, speech, and breathing, some Good Samaritans offered to help him access medical care in different institutions. A computed tomography (CT) scan and a biopsy were requested at one such institution, and performed (Figures 1, 2, and 3). The CT scan showed two large tumors, one in the right maxilla and the other involving the mandible. A chest radiograph did not reveal any metastases, and there was no evidence of any other lesions. A biopsy of the mandible was reported as suggestive of an osteosarcoma. After discussing the histology, CT scan findings and the patient’s clinical status, the doctors referred the patient for palliative care, at the nearest hospice.