Clinicopathological analysis of myxofibrosarcoma of the head

Purpose To study the clinicopathological characteristics and to discuss the differential diagnoses of myxofibrosarcoma(MFS) of the head. Methods The clinical and pathological feature of 8 cases of MFS was evaluated.Among which,6 cases were immunohistochemically studied. Results There were 6 male and 2 female patients,aged between 12 to 58 years(mean 34 years).Five tumors occurred in the nasal cavity or paranasal sinuses,2 in the ear regions,and 1 was located in the orbit.Clinically,most patients presented with nasal obstruction,rhinorrhagia,headache,exophthalmos or a mass in the ear regions.CT scan or MRI usually showed a soft tissue tumor with invasion in the adjacent tissues,associated with bone erosion or destruction.Histologically,the tumors were mainly composed of spindle to stellate-shaped cells deposited in a prominent myxoid matrix.In some cases,hyperchromatic pleomorphic cells and multivacuolated pseudolipoblasts were present. Immunohistochemically,the neoplastic cells were diffusely positive for vimentin and one case expressed α-SMA focally.Follow-up information of 7 patients showed recurrences in two cases at 1 and 2 months after local excision respectively and both died of the disease. Conclusions Myxofibrosarcoma of the head is an aggressive soft tissue sarcoma that has a high rate of local recurrence.Combined modality is recommended to control the relapse of the disease.