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22 April 2015 - Case of the Week #350

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Thanks to Dr. Leart Berdica, University Hospital Center "Mother Theresa" (Albania), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.




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Case of the Week #350

Clinical History:
A 27 year old woman presented with a radiolucent oval mass in the maxilla, which was excised.

Micro images:



What is your diagnosis?































Diagnosis:
Chondroblastic osteosarcoma of maxilla

Discussion:
Osteosarcoma is the most common primary malignant tumor of the jaw, but only 6-10% of all osteosarcomas occur at this site. It usually occurs de novo, but may be associated with radiation therapy, Paget disease of bone or fibrous dysplasia. Typically, those affected are 10-20 years older (mean age 33 to 40 years) than those with osteosarcoma of long bones (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:445, Zhonghua Kou Qiang Yi Xue Za Zhi 2003;38:444, Sarcoma 2013;2013:316123). In the jaw, the most common sites are the mandibular body and maxillary alveolar ridge. These osteosarcomas usually present with nonspecific features, including jaw bone swelling, mucosal ulceration, submucosal mass, pain and nasal obstruction. Radiologic evaluation is difficult due to the anatomic structure of the face and bones and the presence of teeth (Int J Clin Exp Pathol 2014;7:2800).

Histologically, tumor cells produce neoplastic bone. Subtypes include chondroblastic (J Maxillofac Oral Surg 2009;8:290, J Clin Diagn Res 2014;8:288), osteoblastic and fibroblastic.

Clinical, radiographic and histopathologic correlation are recommended. The histologic differential diagnosis includes metastatic tumor (Contemp Clin Dent 2012;3:367, Anticancer Res 2011;31:4485) and chondromyxoid fibroma (J Maxillofac Oral Surg 2015;14 (Suppl 1):87). By fine needle aspiration, tumors may resemble sebaceous carcinoma (Diagn Cytopathol 2011;39:377).

Treatment is usually wide surgical resection, but this is often difficult in jaw osteosarcomas due to anatomic difficulties . Complete resection of maxillary tumors is especially difficult. Tumors often locally recur, but metastases are less likely than with long bone osteosarcoma. Death is usually associated with local tumor extension (Int J Clin Exp Pathol 2014;7:2800).

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