Department of Orthopaedic Surgery, Orthopaedic Oncology, University Hospital of Larissa, Larissa, Greece.
Arch Orthop Trauma Surg. 2012 Jul;132(7):955-61. doi: 10.1007/s00402-012-1510-y. Epub 2012 Apr 10.
Malignant fibrous histiocytoma (MFH) of soft tissue is one of the most common sarcoma in adulthood. However, only a few series have separately studied the clinical behavior and prognosis of this malignancy.
We retrospectively reviewed 61 patients treated for extremity soft tissue high-grade MFH. Four patients had a history of another malignancy and were excluded from analysis. In 12 referred patients with incomplete excision, re-excision of the tumor bed was offered. Clinical and treatment variables were analyzed for their impact on treatment complications, local recurrence (LR), metastatic disease (MD) and overall survival (OS).
Four patients underwent primary amputation. Twenty-three patients necessitated a primary reconstructive procedure for wound closure. Wound healing complication (WHC) developed in 28.3 % of the limb sparing group of patients. LR developed in 11 patients (19.3 %), while 6 of them had second LR. Eighteen patients (31.5 %) developed MD, involving lung at least. Patients who developed MD <12 vs >12 months, died within 19.3 vs 8 months mean time (p < 0.05). Overall survivorship was 66.7 % at 5 years. No statistically significant variables were identified for LR, while multivariate analysis for MD revealed tumor size >5 cm as the only statistically significant variable. For OS, development of MD and age >70 years emerged as independent prognostic factors.
The overall prognosis is poor. LR, although can be managed with tumor re-excision, has high second recurrence rate. Increased tumor size is associated with shorter metastasis-free interval which significantly decreases survival.
软组织恶性纤维组织细胞瘤（MFH）是成人中最常见的肉瘤之一。然而，仅有少数研究分别探讨了这种恶性肿瘤的临床行为和预后。
我们回顾性分析了 61 例接受肢体软组织高级别 MFH 治疗的患者。4 例患者有其他恶性肿瘤病史，被排除在分析之外。在 12 例转诊患者中，对于不完全切除的肿瘤，我们提供肿瘤床的再次切除。分析临床和治疗变量对治疗并发症、局部复发（LR）、转移疾病（MD）和总生存（OS）的影响。
4 例患者接受了初次截肢。23 例患者需要进行原发性重建手术以闭合伤口。保肢组患者中有 28.3%发生了伤口愈合并发症（WHC）。11 例患者发生 LR，其中 6 例发生了第二次 LR。18 例患者（31.5%）发生了 MD，至少累及肺部。发生 MD<12 个月和>12 个月的患者，其平均死亡时间分别为 19.3 个月和 8 个月（p<0.05）。5 年时的总生存率为 66.7%。LR 无统计学显著的变量，而 MD 的多变量分析显示，肿瘤大小>5cm 是唯一有统计学显著的变量。对于 OS，MD 的发生和年龄>70 岁是独立的预后因素。
总体预后较差。LR 虽然可以通过肿瘤再次切除来处理，但复发率较高。肿瘤体积增大与无转移间隔时间缩短相关，这显著降低了生存率。
