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    • Introduction Malignant fibrous histiocytoma MFH is a

    2018-10-22

    Introduction Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma originally described by Ozzello et al in 1963 and O\'Brien and Stout in 1964. It is one of the most common soft-tissue sarcomas among adults, with a peak incidence between 60 and 70 years of age. It has been determined to exhibit large, high-grade, and biological aggressiveness. Most MFHs occur in the extremities (49% in the lower and 19% in the upper extremities), followed by the trunk and retroperitoneum. MFH has been categorized into storiform-pleomorphic, myxoid, giant-cell, and inflammatory types. The tumors involving extremities frequently present as a painless and enlarging mass over a mdm2 inhibitor of months. By contrast, patients with retroperitoneal tumors can exhibit constitutional symptoms, including anorexia, weight loss, fever, and malaise. A multidisciplinary team is necessary to manage malignant soft-tissue tumors, and extremity MFH is the most common type that plastic surgeons treat.
    Materials and methods Clinical and pathologic factors, including age, sex, tumor location, tumor size, excision method, and adjuvant therapy (radiotherapy and chemotherapy), were reviewed and analyzed regarding their influence on the outcome. For data analysis, the age was categorized into the <50-year-old and ≧50-year-old groups, when the tumor was first diagnosed. The tumor size was categorized into groups of <5, 5–10, and >10 cm, according to the largest dimension. All statistical analyses were performed using a statistical software package (PASW for Windows, version 18.0; SPSS Inc, Chicago, IL, USA). Differences of categorical variables were compared with those of Cox regression analysis. Levels of significance were expressed as p values. Actuarial curves for survival and local control were calculated using the Kaplan–Meier method.
    Results Thirteen patients (7 women and 6 men) were included in this study. The mean age of the patients was 57.9 years (33–82 years). The sizes of the clinical tumors ranged from 1.7 to 15 cm in a maximal linear dimension with a mean of 7.3 cm, and 76.9% of tumors occurred in the lower extremities. These lower-extremity tumor sites included the thigh in five cases (50%), the knee in three cases (30%), and the lower leg in two cases (20%). One case with femoral condyle and major vessel involvement received above-knee amputation with a safe margin wider than 5 cm. Two patients with intramuscular MFH received compartmentectomy (Table 1). These three cases (23.1%) were included in the radical excision group. Three patients (23.1%) received a marginal resection and seven patients (53.4%) received a wide excision. All surgical margins were negative for malignant cells. Adjuvant radiotherapy was arranged in only five cases, and chemotherapy was performed in only one case. Concurrent chemoradiotherapy was performed in five cases. The follow-up time for all patients ranged from 3 to 120 months with a mean of 55.4 months. The local recurrence rate was 53.8% (7/13 cases). The statistic correlation between the LDFS and independent prognostic factors is presented in Table 2. There were no significant differences among age, sex, tumor size, location, and adjuvant therapy. Only a marginal excision predicted a significantly high recurrence (Fig. 1). The overall distant-metastasis rate was 30.8% (4/13 cases), and the predominant site for metastasis was the lung. Three cases died of the MFH disease after lung metastasis and one case died of the MFH disease with multiple metastases. Only one patient died of spontaneous intracerebral hemorrhage, which was not related to the MFH tumor. The patients with metastases in the lung had a mean life expectancy of 5.33 months, ranging from 2 to 11 months (Table 1). The 5- and 10-year OS rates were 76.2% and 50.8%, respectively. The statistic correlation between the OS and independent prognostic factors is presented in Table 3. The significant difference affecting the OS was determined by the tumor metastasis (Fig. 2). There were no significant differences among age, sex, tumor size, location, surgery, and adjuvant therapy.