Background: Extraosseous osteosarcoma arising in the head and neck region is an exceptional finding, with a few isolated cases informed in the literature. We report a new case of a large high-grade extraosseous osteosarcoma located in the neck.
Case presentation: A 74 year-old woman with a tumor on the right side of the neck was treated by complete surgical resection. The histopathological diagnosis was osteoblastic extraosseous osteosarcoma. PET-CT scan disclosed no evidence of disease in other areas. Patient received adjuvant radiotherapy and after 3 months of follow-up tumor activity was detected in the cavernous sinus and the patient died of disease one month later.
Conclusion: Extraosseous osteosarcoma in head and neck region is exceptional, and incisional biopsy is the only method to establish a diagnosis and the one to differentiate it from other bone-producing soft tissue tumors. Surgery is the gold standard for treatment of extraosseous osteosarcoma.
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