Grünhagen D J, de Wilt J H W, Verhoef C, van Geel A N, Eggermont A M M
Department of Surgical Oncology, Erasmus MC, Daniel den Hoed Cancer Center, P.O. Box 5201, 3008 AE Rotterdam, The Netherlands.
Eur J Surg Oncol. 2005 Oct;31(8):912-6. doi: 10.1016/j.ejso.2005.07.002.
Desmoid tumours are soft tissue sarcomas with local aggressive behaviour and a high rate of local recurrence after treatment. Although they do not tend to metastasise systemically, the local aggressiveness can lead to situations in which limb-preserving surgery cannot be performed without severe disability. As isolated limb perfusion (ILP) with TNF and melphalan has proven to be extremely effective in the treatment of soft tissue sarcoma, we studied its potential in locally advanced extremity desmoid tumours.
Prospectively maintained database in a tertiary referral centre. Between 1991 and 2003, 12 ILP procedures were performed in 11 patients for locally advanced desmoid tumours. Local surgical therapy with preservation of limb function was impossible in all patients due to large or multifocal tumours, multiple recurrences or extensive previous treatment. Perfusions were performed with 4-3mg TNF and 10-13 mg/l limb volume melphalan form leg and arm perfusions, respectively.
Overall response rate was 75%: Two complete responses were recorded (17%) and seven patients had a partial response (58%). Amputation could be avoided in all cases. Local control was obtained after 10/12 ILPs and in the other two patients through repeat ILP and systemic chemotherapy, thus leading to an overall local control rate of 100%. Local toxicity was mild and systemic toxicity was absent in all patients.
ILP is a very effective treatment option in the multimodality treatment of limb desmoid tumours. It should be considered in patients with aggressive and disabling disease where resection without important functional sacrifice is impossible.
硬纤维瘤是一种软组织肉瘤，具有局部侵袭性，治疗后局部复发率高。尽管它们一般不会发生全身转移，但局部侵袭性可能导致在不造成严重残疾的情况下无法进行保肢手术的情况。由于已证明肿瘤坏死因子（TNF）和马法兰的隔离肢体灌注（ILP）在软组织肉瘤治疗中极为有效，我们研究了其在局部晚期肢体硬纤维瘤中的应用潜力。
在一家三级转诊中心前瞻性维护数据库。1991年至2003年间，对11例患者进行了12次ILP手术，用于治疗局部晚期硬纤维瘤。由于肿瘤体积大或多灶性、多次复发或既往治疗广泛，所有患者均无法进行保留肢体功能的局部手术治疗。腿部和手臂灌注分别使用4 - 3mg TNF和10 - 13mg/l肢体体积的马法兰进行灌注。
总体缓解率为75%：记录到2例完全缓解（17%），7例患者部分缓解（58%）。所有病例均避免了截肢。12次ILP中有10次实现了局部控制，另外2例患者通过重复ILP和全身化疗实现了局部控制，总体局部控制率达到100%。所有患者局部毒性轻微，无全身毒性。
ILP是肢体硬纤维瘤多模式治疗中非常有效的治疗选择。对于患有侵袭性且致残性疾病、无法在不严重牺牲功能的情况下进行切除的患者，应考虑采用ILP治疗。
