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Malignant fibrous histiocytoma of the extremities and trunk: an institutional review.

机译:四肢和躯干的恶性纤维组织细胞瘤:一项机构审查。

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BACKGROUND: Malignant fibrous histiocytoma (MFH) is the most common subtype of soft-tissue sarcoma. Detailed understanding of this tumor type may lead to improved therapeutic strategies. METHODS: An institutional review was performed on all patients with primary MFH of the extremities and trunk operated on between 1988 and 2000. RESULTS: Ninety-seven patients with histologically confirmed MFH (G1, n=8; G2, n=25; G3, n=64) were analyzed. Local recurrence was 31% after a median of 13 months. Distant metastases occurred in 29 patients (30%). After a median follow-up of 4.5 years, 54 patients were alive without evidence of disease; median survival time was 84 months at a cumulative 5-year survival rate of 70%. Tumor size significantly influenced disease-free survival (T2 vs T1, P<.01, risk ratio [RR] 6.0), as did tumor depth (subfascial tumors, P<.01, RR 3.1) and presence of positive lymph nodes (P=.02, RR 6.9). Positive microscopic margins and subfascial tumors were associated with an increased local recurrence rate (RR 4.8, P<.001 and RR 3.5, P=.02, respectively). Significant multivariate risk factors of distant metastases were tumor size, depth, and grade. Though not performed in a randomized fashion, a subgroup analysis indicated that adjuvant radiation therapy significantly reduced local tumor failure. CONCLUSION: We conclude that aggressive, albeit limb-preserving resection of MFH, should be performed at initial operation to minimize risk of local recurrence; a strict follow-up especially of subfascial tumors should be performed.
机译:背景:恶性纤维组织细胞瘤(MFH)是软组织肉瘤最常见的亚型。对这种肿瘤类型的详细了解可能会导致改善治疗策略。方法:对所有在1988年至2000年期间手术的四肢和躯干原发性MFH患者进行了机构审查。结果:97例经组织学证实为MFH的患者(G1,n = 8; G2,n = 25; G3, n = 64)。中位13个月后,局部复发率为31%。 29例患者发生远处转移(30%)。中位随访4.5年后,有54名患者还活着,没有疾病迹象;中位生存时间为84个月,累计5年生存率为70%。肿瘤大小显着影响无病生存期(T2 vs T1,P <.01,风险比[RR] 6.0),肿瘤深度(筋膜下肿瘤,P <.01,RR 3.1)和淋巴结阳性(P = .02,RR 6.9)。显微镜下切缘阳性和筋膜下肿瘤与局部复发率升高相关(分别为RR 4.8,P <.001和RR 3.5,P = .02)。远处转移的重要多因素危险因素是肿瘤大小,深度和等级。尽管未以随机方式进行,但亚组分析表明辅助放疗显着降低了局部肿瘤衰竭。结论:我们得出结论,应在初次手术时进行积极的,尽管保留肢体的MFH切除,以最大程度地减少局部复发的风险。应当严格随访,尤其是筋膜下肿瘤。

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