Abstract:
The European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS-05 (EUDRACT 2005-001139-31) and in MTS-2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.0-18.8). Local therapy was initial primary surgery in seven cases, and five patients received systemic therapy. No patients received radiotherapy. After a median follow-up of 50 months (range, 6-176) for living patients, nine patients remain alive off therapy and two died. Five-year progression free and overall survivals are, respectively, 77.1% (95% confidence interval [CI]: 34.5-93.9) and 74.1% (95% CI: 28.1-93.0).
摘要:
欧洲儿童软组织肉瘤研究组分析了所有在NRSTS-05(EUDRACT2005-001139-31)和MTS-2008(NCT00379457)研究中前瞻性登记的上皮样血管内皮瘤儿童:10例局限性疾病和1例转移性疾病。中位年龄为14.3岁(范围,9.0-18.8)。局部治疗是7例初次手术,5例患者接受了全身治疗。无患者接受放疗。在中位随访50个月后(范围,6-176)对于活着的病人,9名患者在接受治疗后仍然存活,2人死亡。五年无进展和总体生存率是,分别,77.1%(95%置信区间[CI]:34.5-93.9)和74.1%(95%CI:28.1-93.0)。