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Primary osteogenic sarcoma of the maxilla.

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A 70-year-old man presented with a large mass on his right upper jaw (figure, A). He had had a lesion removed from his right maxillary alveolus 7 months previously with a pathologic diagnosis of benign ossifying fibroma. Computed tomography (CT) of the facial bones demonstrated a hyperdense, destructive mass of the right maxillary bone, extending to the midline medially and laterally into the soft tissues of the cheek (figure, B).

A maxillectomy with preservation of the floor of the orbit and repair with a split-thickness graft was performed. The maxillary bone showed an irregular, hard tumor mass measuring 7 x 5 x 5 cm with a central crater-shaped ulcer, 2.5 cm diameter, in the overlying mucosa. The tumor histology was consistent with conventional osteogenic sarcoma, grade 2 to 3 (on a scale of 4) with focal chondroblastic differentiation (figure, C, D, and E). The anterior and posterior resection margins were focally involved by the tumor.

Postoperatively, intensity-modulated radiation therapy was given. Tumor recurrence was recently noted (38 months after resection) with no evidence of distant metastases. The patient is scheduled for additional surgery.

Primary osteogenic sarcomas occur most frequently in the long bones. However, approximately 10% occur in the head and neck, most commonly in the mandible, followed by the alveolar ridge of the maxilla, with similar gender distribution. (1,2) There is a wide age range, with a peak incidence in the third and fourth decades of life, unlike osteosarcoma of the long bones, which occurs mostly in the second decade of life. The most frequent symptoms are swelling of the affected jaw and pain. (3) On radiographic imaging the tumor may be osteolytic or osteoblastic, and a sunburst appearance is seen in 30% to 55% of cases. (4,5)

Osteosarcoma of the jaw is an aggressive malignancy with a high rate of local recurrence. However, the risk of distant metastases is lower than that of osteosarcoma of the long bones. The overall 5-year survival rate is between 6 and 85%. (6)

Treatment of osteosarcoma of the head and neck presents a therapeutic challenge and is different from treatment of osteosarcoma of the extremities because of the sensitivity of its anatomic location, poor tolerance to conventional treatment, and lack of a standardized treatment protocol. (3)

Radical surgery with complete tumor resection is the treatment of choice. (1,3,4,6,7) Negative resection margins are of paramount importance for optimizing survival. (2) However, achieving clear surgical margins may be difficult because of anatomic constraints. Adjuvant radiation-chemotherapy may be beneficial in the local control of residual tumor (if negative margins can not be obtained) and in improving the cure rate and disease-free survival.

Osteosarcoma is generally believed to be relatively radioresistant, requiring doses greater than 60 Gy to be effective. (6) The proximity of the tumor to several vital and radiosensitive structures imposes limitations on the total radiation dose that can be delivered, to respect the tolerance of the surrounding tissues and avoid serious late morbidity of radiotherapy. Modern radiation techniques, such as 3D conformal radiotherapy and intensity-modulation radiotherapy are currently used (as in our case) to overcome these dose-limiting factors, to achieve effective disease control, and to reduce treatment-related damage to vital structures.

References

(1.) Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol 2003;15(3):239-52.

(2.) Canadian Society of Otolaryngology Study Group. Osteogenic sarcoma of the mandible and maxilla: A Canadian review (1980-2000). J Otolaryngol 2004;33(3):139-44.

(3.) Laskar S, Basu A, Muckaden MA, et al. Osteosarcoma of the head and neck region: Lessons learned from a single-institution experience of 50 patients. Head Neck 2008;30(8):1020-6.

(4.) Fernandes R, Nikitakis NG, Pazoki A, Ord R. Osteogenic sarcoma of the jaw: A 10-year experience. J Oral Maxillofac Surg 2007; 65(7):1286-91.

(5.) Nakayama E, Sugiura K, Ishibashi H, et al. The clinical and diagnostic imaging findings of osteosarcoma of the jaw. Dentomaxillofac Radiol 2005;34(3):182-8.

(6.) Oda D, Bavisotto LM, Schmidt RA, et al. Head and neck osteosarcoma at the University of Washington. Head Neck 1997; 19(6):513-23.

(7.) Bennett JH, Thomas G, Evans AW, Speight PM. Osteosarcoma of the jaws: A 30-year retrospective review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90(3):323-32.

From the Department of Pathology (Dr. Makary and Dr. Shuja), the Section of Head and Neck Surgery (Dr. Fernandes and Dr. Goldman), and the Department of Radiation Oncology (Dr. Malyapa), University of Florida College of Medicine, Jacksonville.

Raafat Makary, MD; Sania Shuja, MD; Ruis Fernandes, DMD, MD; Robert Malyapa, MD; Nelson Goldman, MD
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Title Annotation:HEAD AND NECK CLINIC
Author:Makary, Raafat; Shuja, Sania; Fernandes, Ruis; Malyapa, Robert; Goldman, Nelson
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Oct 1, 2010
Words:770
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