Hoffmann C, Jabar S, Ahrens S, Rödl R, Rübe C, Winkelmann W, Dunst J, Jürgens H
Universitäts-Kinderklinik Münster.
Klin Padiatr. 1995 Jul-Aug;207(4):151-7. doi: 10.1055/s-2008-1046532.
In this retrospective analysis, data of 52 patients with Ewing's sarcoma or PNET with a pathological fracture in the area of the primary tumor were evaluated. All patients were treated according to the trials CESS 81, CESS 86 P, CESS 86, CESS 91 P and EICESS 92 of the German Society of Pediatric Oncology and Haematology (GPOH). At the date of evaluation (15. September 1994) all patients had completed treatment and had been under observation for at least one year following diagnosis. The median follow-up time was 28 months. 22 patients were female, 30 male. The median age was 12 years. 75% of primary tumors had a volume of > or = 100 ml. 30 patients presented with fractures in proximal, 12 in central and 10 in distal parts of the skeleton. 10 patients had primary metastases. The histological definition was Ewing's sarcoma (including atypical Ewing's sarcoma) in 43 patients, PNET in 8 and small-cell osteosarcoma in 1 patient. For local therapy the patients underwent surgery, definitive radiotherapy or a combination of both. The percentage of primary metastases in the group of the patients with pathological fractures is comparable to the whole reference group. The present analysis focuses on those patients with pathological fractures who had no metastases at diagnosis. The relapse-free survival of patients with a pathological fracture and no primary metastases is 58%, the overall survival 65%. These rates are similar to those of the reference group of protocol patients without pathological fractures at diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
在这项回顾性分析中,对52例原发性肿瘤部位发生病理性骨折的尤因肉瘤或原始神经外胚层肿瘤(PNET)患者的数据进行了评估。所有患者均按照德国儿科肿瘤学和血液学协会(GPOH)的CESS 81、CESS 86 P、CESS 86、CESS 91 P和EICESS 92试验进行治疗。在评估日期(1994年9月15日),所有患者均已完成治疗,且自诊断后已接受至少一年的观察。中位随访时间为28个月。22例为女性,30例为男性。中位年龄为12岁。75%的原发性肿瘤体积≥100 ml。30例患者的骨折发生在骨骼近端,12例在中部,10例在远端。10例患者有原发性转移。组织学诊断为尤因肉瘤(包括非典型尤因肉瘤)43例,PNET 8例,小细胞骨肉瘤1例。对于局部治疗,患者接受了手术、根治性放疗或两者联合。病理性骨折患者组中原发性转移的百分比与整个参照组相当。本分析聚焦于那些诊断时无转移的病理性骨折患者。病理性骨折且无原发性转移患者的无复发生存率为58%,总生存率为65%。这些比率与诊断时无病理性骨折的方案患者参照组相似。(摘要截选至250词)