Head and neck myxofibrosarcoma: A case report and review of the literature

Introduction: Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, peaking in the seventh decade, and it is mainly encountered in the lower extremities. Myxofibrosarcoma of the head and neck are extremely rare. To the best of our knowledge, only 19 cases have been described in the head and neck so far. This is a literature review and retrospective chart review of our experience in head and neck myxofibrosarcoma treatment in our department. Case presentation: In this case report we describe a 35-year-old Caucasian man who presented the first case of myxofibrosarcoma arising from the pterygopalatine fossa. The peculiar anatomical location and the extent in the midcheek region make this case a hard "challenge" for the surgeon, in order to guarantee wide surgical margins of resection. A total right maxillectomy was accomplished by means of the Weber-Ferguson approach, preserving the orbital floor. The excised portion was reconstructed using the free rectus abdominis myocutaneous flap. Postoperative radiotherapy was given to the area adjacent to the lesion, with a total dose of 60Gy. No relapse occurred in the 27-month postoperative follow-up. Conclusions: The case described suggests the importance of combined surgical and adjuvant radiotherapy to avoid local and distant recurrences of the tumor. In our opinion, combined surgical and adjuvant radiotherapy followed by close clinical observation to search for a metastatic disease is advisable in all cases. Further studies are needed to confirm the efficacy of combined radio-chemotherapy for head and neck myxofibrosarcoma in terms of long-term disease-free survival.