Parosteal osteosarcoma of the maxilla: a case report
Parosteal osteosarcomas are usually low-grade tumors, however, sometimes they transform to high-grade tumors, which is named dedifferentiation. This phenomenon has been reported in long bones. Published in the Journal of Medical Case Reports, the authors report a first case of dedifferentiated parosteal osteosarcoma of the head and neck region.

A 45-year-old woman with a refractory bone lesion in the maxilla presented to the hospital. A biopsy revealed atypical spindle cell proliferation involving dedifferentiated high-grade component, which was diagnosed as dedifferentiated parosteal osteosarcoma. Three cycles of neoadjuvant chemotherapy using ifosfamide and pirarubicin were performed followed by sub-total maxillectomy.

Histopathological results showed that neoadjuvant chemotherapy was effective for high-grade component. The decision to perform adjuvant chemotherapy (cisplatin and pirarubicin) was made because distant metastasis has been reported, even in cases with dedifferentiated parosteal osteosarcoma in which complete necrosis of high-grade component was achieved due to neoadjuvant chemotherapy. There was no recurrence 15 months after surgery.

Clinical Pearls:-
Dedifferentiated parosteal osteosarcoma can occur in the head and neck region. Chemotherapy including anthracycline anticancer agent could be effective for high-grade component of dedifferentiated parosteal osteosarcoma.

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