Myofibrosarcoma-two cases report and review of literature / 中国矫形外科杂志

ABSTRACT

[Objective]To investigate the clinical and histopathological characteristics of myofibrosarcoma,and study its treatment and prognosis.[Method]The clinical features,histopathological characteristics,diagnosis,treatment and prognosis of two rare myofibrosarcoma patients were observed,and relative literatures were reviewed.[Result]After biopsy,the frist patient was treated with amputation in her middle thigh.postoperative chemotherapy was performed in the local hospital,but the drug and its dose didn't know in detail.one year latter,the patient died because of lung metastasis.The second patient was treated with tumor resection,but three months latter,the tumor recurred and was resected again,and the patient accepted chemotherapy,eleven nonthns latter,lung metastasis was found.then the patient died.About 54 patients with myofibrosarcoma were found in the literatures published in recent years.This neoplasms often involved the head and neck region,trunk and extremities in male patient A expandable and painless mass is the chief clinical features.Myofibrosarcoma can be identified by its specific morphologic features and positive myoid markers.[Conclusion]Myofibrosarcoma is a rare spindle cell neoplasm,Its morbidity dosen't know in detail.When making diagnisis,it is important to distinguished from synovial sarcoma,fibrosarcoma and leiomyosarcoma et al,Surgical resection is the mainstay of therapy,but the result of chemotherapy is not affirmed,The tumor is lower grade malignant one,and is invadable.The rate of local recurrence is high,and distant metastasis could be found.Its prognosis is not affirmed.