Gnathic osteosarcomas: Review of literature and report of two cases in maxilla

Abstract

Primary neoplasms of the skeleton are rare, accounting for 0.2% of overall human tumor burden. Osteosarcoma (OS) accounts for 15–35% of all primary bone tumors, while gnathic osteosarcomas (GOS) represent 4–8% of all osteosarcomas. GOS shows a predilection for men, a peak incidence of 33 years, and affects the mandible more than the maxilla. We review the scientific literature for a better understanding of the clinical, radiographic, and histopathological features of GOS, along with its etiology, staging, treatment protocol, prognosis, and survival. Evidence from molecular research suggests that it is a differentiation disease that disrupts osteoblasts differentiation from mesenchymal stem cells. The classical radiographic finding of a “sunburst” appearance is appreciated only in 50% of GOS. The universally accepted staging system is not commonly used due to the rarity with which they metastasize to the regional lymph nodes. A number of distinct histopathological subtypes have been described, of which osteoblastic GOS are most common. The treatment protocol is multimodal consisting of preoperative chemotherapy followed by surgery and postoperative chemotherapy, and has a 60-70% five-year survival rate. We present two case reports of osteosarcoma involving the maxillary that were initially misdiagnosed as peripheral giant cell granuloma and osteoma of the maxilla, respectively. These case reports demonstrate the diverse clinical, radiographic, and histopathological features that can be encountered in GOS.

Chondroblastic Osteosarcoma in A 13 Year Old Child

“…6 OS is characterized by production of osteoid tissue by mesenchymal tumor cells. 3 It is the second most common malignant bone tumor after multiple myeloma. 3 OS accounts for 15-35% of all primary bone tumors followed by chondrosarcoma and Ewing's sarcoma.…”

“…3 It is the second most common malignant bone tumor after multiple myeloma. 3 OS accounts for 15-35% of all primary bone tumors followed by chondrosarcoma and Ewing's sarcoma. 1 OS of jaw bones are rare and account for 5-6% of all osteosarcomas with an estimated incidence of 0.07 cases per 100,000 per year.…”

“…It is the most common non-hemopoietic primary malignant bone tumor of children and adolescence. 3 Osteosarcomas of jaw are diagnosed two decades and later than sarcomas of long bone with a peak incidence between 20-40 years. Osteosarcomas of the head and neck originate from soft tissues and have lower metastatic rate than osteosarcomas of long bones.…”

“…2 OS of jaw occurs mostly in males with a ratio of 1.5:1 and peak incidence is observed in 3-4 th decade. 3 Common site of occurrence is body of mandible followed by alveolar ridge of maxilla and…”

Osteosarcoma of the Jaw: Classification, Diagnosis and Treatment

“…JOS is relatively rare and the majority of them arise de novo but some of them may develop in bone afected by Paget's disease, ibrous dysplasia, bone infarcts, chronic osteomyelitis, trauma, viral infection, exposure to high-dose radiation, metallic implants, joint prostheses in genetic syndromes such as Li-Fraumeni syndrome, hereditary retinoblastoma and RTx [36].…”