Koscielniak E, Jürgens H, Winkler K, Bürger D, Herbst M, Keim M, Bernhard G, Treuner J
Department of Oncology/Haematology, Olga Hospital, Stuttgart, Germany.
Cancer. 1992 Nov 15;70(10):2557-67. doi: 10.1002/1097-0142(19921115)70:10<2557::aid-cncr2820701027>3.0.co;2-8.
In the first German soft tissue sarcoma (STS) study, CWS-81, 344 patients younger than 19 years of age who had previously untreated soft tissue sarcoma were studied. For this analysis, there were 218 patients with chemosensitive STS (Group A: rhabdomyosarcoma [RMS], synovial sarcoma, extraosseous Ewing sarcoma, leiomyosarcoma, undifferentiated sarcoma, and malignant peripheral neuroectodermal tumor) who could be studied for a minimum potential follow-up time of 6 years.
A staging system based on the postoperative extent of the disease was used. The chemotherapy for Stage I-III disease consisted of vincristine, dactinomycin, cyclophosphamide, and doxorubicin (VACA). Patients with metastatic disease and patients with Stage III disease who failed to respond to VACA were given ifosfamide instead of cyclophosphamide. The definitive procedure for local tumor control (either no radiation exposure, 40 Gy, or 50 Gy) for patients with Stage II-III disease depended on the tumor status at second-look surgery after 16 weeks of chemotherapy.
The rates of disease-free survival (DFS) and survival after 5 years was 61% +/- 4% and 57% +/- 4%, respectively, in group A; for patients with nonmetastatic tumors (Stages I-III), the rates were 69% +/- 4% and 72% +/- 4, respectively. Patients with nonmetastatic rhabdomyosarcoma had a similar prognosis: the survival rate was 73% +/- 4%, and the DFS rate was 68% +/- 4%. There was no difference in prognosis between patients with Stage I and and those with Stage II disease (DFS rate, 88% +/- 5% and 88% +/- 6%, respectively). The DFS rate for patients with Stage III disease was 54% +/- 5% and for those with Stage IV, 11% +/- 5%. Lack of local tumor control was the primary cause of therapy failure: 10% of patients with localized disease did not achieve complete remission, whereas 18% who were in complete remission experienced local relapse. The most important prognostic factors were tumor size (P = 0.005) and the degree of tumor regression after primary chemotherapy (P = 0.007). The prognosis also differed according to primary site: paratesticular tumors had the best prognosis, whereas tumors located in the parameningeal regions of the head and neck had the worst prognosis (DFS rate, 96% +/- 4% versus 49% +/- 7%, respectively).
The following conclusions were drawn from the CWS-81 study: (1) intensive chemotherapy (VACA for 35 weeks) provides long-term control for most patients with Stage I-II disease; (2) patients with primary unresectable tumors (i.e., Stage III) who achieve complete remission with chemotherapy alone have the same prognosis as patients with postoperative disease of Stages I and II; (3) tumor size and the degree of tumor regression after primary chemotherapy influence outcome and thus can be used as a basis for risk-adapted therapy.
在德国首个软组织肉瘤（STS）研究CWS - 81中，对344例19岁以下先前未经治疗的软组织肉瘤患者进行了研究。在本次分析中，有218例对化疗敏感的STS患者（A组：横纹肌肉瘤[RMS]、滑膜肉瘤、骨外尤文肉瘤、平滑肌肉瘤、未分化肉瘤和恶性外周神经外胚层肿瘤）可进行至少6年的潜在随访研究。
采用基于疾病术后范围的分期系统。I - III期疾病的化疗方案为长春新碱、放线菌素D、环磷酰胺和阿霉素（VACA）。转移性疾病患者以及对VACA无反应的III期疾病患者，用异环磷酰胺替代环磷酰胺。II - III期疾病患者局部肿瘤控制的确定性程序（无放疗、40 Gy或50 Gy）取决于化疗16周后二次探查手术时的肿瘤状态。
A组5年无病生存率（DFS）和总生存率分别为61%±4%和57%±4%；对于非转移性肿瘤（I - III期）患者，这两个比率分别为69%±4%和72%±4%。非转移性横纹肌肉瘤患者预后相似：生存率为73%±4%，DFS率为68%±4%。I期和II期患者预后无差异（DFS率分别为88%±5%和88%±6%）。III期疾病患者的DFS率为54%±5%，IV期患者为11%±5%。局部肿瘤控制不佳是治疗失败的主要原因：10%的局限性疾病患者未实现完全缓解，而18%完全缓解的患者出现局部复发。最重要的预后因素是肿瘤大小（P = 0.005）和初始化疗后肿瘤消退程度（P = 0.007）。预后也因原发部位而异：睾丸旁肿瘤预后最佳，而位于头颈部脑膜旁区域的肿瘤预后最差（DFS率分别为96%±4%和49%±7%）。
从CWS - 81研究得出以下结论：（1）强化化疗（VACA方案35周）可为大多数I - II期疾病患者提供长期控制；（2）初始不可切除肿瘤（即III期）患者仅通过化疗实现完全缓解，其预后与I期和II期术后疾病患者相同；（3）肿瘤大小和初始化疗后肿瘤消退程度影响预后，因此可作为风险适应性治疗的依据。
