Chondroblastic osteosarcoma of maxilla: a case report and review of literature

Abstract

Craniofacial osteosarcoma is a relatively rare entity comprising about 6.5-7% of all osteosarcomas, whereas it is reported to be one of the most common malignancies of long bones. We present a case of chondroblastic variant of osteosarcoma (COS) of the maxilla in an 18 year-old-girl. She was treated with a subtotal maxillectomy and later referred for adjuvant chemotherapy. The relevant review of literature with regard to clinical, radiographic findings, histopathological similarities to Chondrosarcoma (CS), role of Immunohistochemistry (IHC) and management modalities of Osteosarcoma of jaw bones (OSJ) in comparison with Osteosarcoma of the long bones (OSL) are being presented in this report.

Osteosarcoma of the jaws: a review of literature and a case report on synchronous multicentric osteosarcomas

“…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].…”

Atypical Presentation of a Maxillary Chondroblastic Osteosarcoma and Complex Management

“…OS is histologically characterised by the presence of malignant and undifferentiated cellular stroma that produce neoplastic osteoid or primitive bone [1,4]. The histological subtype of OS is classified according to the predominant matrix material present, depending on whether it is osteoid (osteoblastic), cartilaginous (chondroblastic) or minimal matrix (fibroblastic) [10].…”

“…It is essential that chondroblastic OS is differentiated from chondrosarcoma, as the latter is associated with a better prognosis and rarely occurs in adolescents or young adults [4,11]. Due to the presence of cartilaginous tissue, small specimens can mislead pathologists into diagnosing a chondrosarcoma.…”

“…Due to the presence of cartilaginous tissue, small specimens can mislead pathologists into diagnosing a chondrosarcoma. This is due to the lack of osteoid production that is visible during analysis when a specimen is obtained using techniques, such as incisional biopsies [4,12]. The false diagnosis rate is believed to be as high as 44% [13].…”

“…Involvement of the craniofacial skeleton remains relatively rare, with an estimated incidence of less than 1 per million and is found only in 6-10% of all OS cases [3]. Long bone OS and craniofacial OS share microscopic features; however, they are considered as separate entities [4]. OS of the long bones age of onset tends to be a bimodal distribution from adolescence and the fifth decade, the most common cause of mortality is distant metastasis, painful swelling as well as the rarity of neuroparaesthesia amongst other factors [5,6].…”