Kirschner M J, Sauer R
Strahlentherapeutische Klinik und Poliklinik, Universität Erlangen-Nürnberg.
Strahlenther Onkol. 1993 Feb;169(2):77-82.
Aggressive fibromatoses are benign tumors which derive from connective tissues in muscles, tendons, joints and scars. They are characterized by a low mitotic index and present without major cellular changes. The incidence is low and reaches 0.4 per 100,000. The classification in extra-abdominal, intra-abdominal and abdominal wall tumors has prognostic significance for the therapeutic decision. Extra-abdominal tumors have the highest relapse rate with about two-thirds recurring even after R0 surgical resection. In this meta-analysis, 698 published cases are reviewed and analyzed with respect to the previous surgery and the therapeutic impact of postoperative radiotherapy. After an R1 surgical resection radiotherapy significantly decreases the risk for loco-regional relapse in about 40%. Radiotherapy is also recommended after an R0 surgical resection with safety margins less than 2 cm. A total radiotherapy dose of 50 Gy seems to be sufficient, whereas radiotherapy doses beyond 60 Gy provide no additional control. Following an R2 resection up to 28% of these tumors may not progress or even show a complete remission. A wait-and-see policy is justified for R1-2 resected lesions in the abdominal wall, especially if severe treatment sequelae have to be expected. The role of chemotherapy still remains questionable.
侵袭性纤维瘤病是一种起源于肌肉、肌腱、关节和瘢痕结缔组织的良性肿瘤。其特点是有丝分裂指数低，且无明显细胞变化。发病率较低，每10万人中约有0.4例。腹外、腹内和腹壁肿瘤的分类对治疗决策具有预后意义。腹外肿瘤的复发率最高，即使在R0手术切除后，约三分之二的患者仍会复发。在这项荟萃分析中，对698例已发表的病例进行了回顾和分析，涉及既往手术情况以及术后放疗的治疗效果。在R1手术切除后，放疗可使局部区域复发风险显著降低约40%。对于切缘小于2 cm的R0手术切除，也建议进行放疗。总放疗剂量50 Gy似乎就足够了，而超过60 Gy的放疗剂量并不能提供额外的控制效果。在R2切除后，高达28%的此类肿瘤可能不会进展，甚至可能完全缓解。对于腹壁R1-2切除的病变，尤其是如果预期会出现严重的治疗后遗症，采取观察等待的策略是合理的。化疗的作用仍存在疑问。
