The Rina Zaizov Hematology-Oncology Division, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.
Sackler faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Cancer Chemother Pharmacol. 2019 May;83(5):859-866. doi: 10.1007/s00280-019-03789-3. Epub 2019 Feb 15.
We report the unexpected absence of early relapse (before 30 months) in 24 consecutive patients with isolated limb primary Ewing sarcoma treated with an intensified pilot protocol, SCMCIE94.
Clinical data for the study were collected retrospectively from the patient files. The protocol included 6 courses of chemotherapy, split radiation, and limb salvage surgery. This SCMCIE94 protocol had been used in almost all the patients described in an earlier report, in whom those with non-pelvic isolated tumors and low/absent CD56 expression in Ewing sarcoma tumor cells were all long-term survivors.
The 5-year (10-year) event-free survival rate for the patients with isolated limb primary Ewing sarcoma was 78.95 ± 8.3% (68.6 ± 10.0%) and the overall survival rate was 90.7 ± 6.2% (71.1 ± 11.2%). There were no relapses before 30 months in any of these patients.
The intensified SCMCIE94 pilot protocol has been shown previously to cure patients with localized CD56-negative non-pelvic Ewing sarcoma. The present study shows that among all patients with localized extremity disease who were treated with this protocol, there were no cases of early relapse. Although our cohort was small, the difference in results from studies using other protocols is so striking, that it would seem reasonable to assume it is attributable to the changes made in the protocol itself rather than risk factors. Late relapses of isolated limb CD56-positive Ewing sarcoma suggest minimal residual disease warranting additional therapeutic approaches such as autologous stem cell rescue after Busulfan Melfelan.
我们报告了 24 例连续孤立肢体原发性尤文肉瘤患者在接受强化试验方案 SCMCIE94 治疗后,意外未出现早期复发(30 个月前)。
研究的临床数据从患者档案中回顾性收集。该方案包括 6 个疗程的化疗、分割放疗和保肢手术。该 SCMCIE94 方案几乎用于之前报道的所有患者,其中所有具有非骨盆孤立性肿瘤和尤文肉瘤肿瘤细胞中 CD56 低表达/缺失的患者均为长期幸存者。
孤立肢体原发性尤文肉瘤患者的 5 年(10 年)无事件生存率为 78.95±8.3%(68.6±10.0%),总生存率为 90.7±6.2%(71.1±11.2%)。这些患者中无一例在 30 个月前复发。
之前的研究表明,强化的 SCMCIE94 试验方案已治愈局部 CD56 阴性非骨盆尤文肉瘤患者。本研究表明,在接受该方案治疗的所有局限性肢体疾病患者中,均未出现早期复发。尽管我们的队列规模较小,但与使用其他方案的研究结果差异如此显著,以至于有理由认为这归因于方案本身的改变,而不是风险因素。孤立肢体 CD56 阳性尤文肉瘤的晚期复发表明存在微小残留疾病,需要额外的治疗方法,如博舒替尼和马法兰后自体干细胞解救。