Radiation-induced osteosarcoma of the maxillary sinus.

A 41-year-old man presented with an osteosarcoma involving the right maxillary sinus. The patient had a history of a right maxillary embryonal rhabdomyosarcoma that had been treated with chemoradiation 8 years earlier; this suggested a current diagnosis of radiation-induced osteosarcoma (RIO). Findings on the head and neck examination were normal except for cranial nerve V2 hypoesthesia. Magnetic resonance imaging (MRI) detected an irregular enhancing mass that involved the right maxillary sinus and extended through the floor of the orbit and into the right ethmoid sinus (figure 1). Because of the tumor's proximity to the orbit, the patient opted for chemotherapy prior to surgical intervention in an attempt to avoid orbital exenteration.

[FIGURE 1 OMITTED]

After chemotherapy, the patient underwent a right subtotal maxillectomy via a modified Weber-Ferguson incision (figure 2). Gross tumor had not invaded the periorbita, microscopic disease was ruled out by frozen-section analysis, and the globe was spared. The floor of the orbit was reconstructed with a Medpor orbital implant. A titanium mesh with overlying Mimix hydroxyapatite cement was used to reconstruct the orbital rim (figure 3). The palatal defect was obturated with a prosthesis.

[FIGURES 2-3 OMITTED]

Postoperatively, the patient underwent additional chemotherapy, and at follow-up 26 months later, he remained free of disease.

Osteosarcomas are determined to be (or not be) radiation-induced on the basis of criteria first developed in 1948 by Cahan et al (1) and later modified by Arlen and Marcove. (2) The four criteria are (1) a history of primary radiation for a benign process or a malignancy without osteoblastic activity, (2) the presence of a bony sarcoma arising within the path of the radiation beam, (3) a long asymptomatic period, and (4) the presence of histologically proven osteosarcoma.

RIO of the maxilla, which is rare, has been described after treatment for retinoblastoma, nasopharyngeal carcinoma, and head and neck squamous cell carcinoma. In 2005, Wei-wei et al reported 15 cases of RIO following radiation treatment in 40,719 cases of nasopharyngeal carcinoma; 5 of these RIOs occurred in the maxilla, 7 in the mandible, and 3 in the nasal cavity. (3) The mean time to development of RIO after radiation was 13 years. Negative prognostic factors for survival in these patients were male sex and a greater soft-tissue component vs. bone within the tumor. Estimated 1- and 2-year survival rates were 60 and 24%, respectively.

Treatment of RIO of the maxilla is primarily surgical. In 1999, Tabone et al found that complete surgical resection was a statistically significant prognostic factor in children with RIO. (4) Although the role of chemotherapy is not clearly defined, adjuvant chemotherapy may improve overall survival.

References

(1.) Cahan WG, Woodard HQ, Higinbotham NL, et al. Sarcoma arising in irradiated bone: Report of eleven cases. Cancer 1948; 1:3-29.

(2.) Arlen M, Higinbotham NL, Huvos AG, et al. Radiatin-induced sarcoma of bone. Cancer 1971;28(5):1087-99.

(3.) Wei-wei L, Qiu-liang W, Guo-hao W, et al. Clinicopathologic features, treatment, and prognosis of postirradiation osteosarcoma in patients with nasopharyngeal cancer. Laryngoscope 2005; 115(9): 1574-9.

(4.) Tabone MD, Terrier P, Pacquement H, et al. Outcome of radiation-related osteosarcoma after treatment of childhood and adolescent cancer: A study of 23 cases. J Clin Oncol 1999;17(9):2789-95.