儿童上皮样血管内皮细胞瘤：欧洲儿科软组织肉瘤研究组的经验。
Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience.
机构信息
SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France.
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
出版信息
Pediatr Blood Cancer. 2022 Oct;69(10):e29882. doi: 10.1002/pbc.29882. Epub 2022 Jul 16.
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).
欧洲儿科软组织肉瘤研究组对所有在 NRSTS-05（EUDRACT 2005-001139-31）和 MTS-2008（NCT00379457）研究中前瞻性登记的局限性和一例转移性上皮样血管内皮细胞瘤患儿进行了分析：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）。局部治疗为初始原发手术 7 例，5 例患者接受了系统治疗。无患者接受放疗。在可评估的生存患者中，中位随访时间为 50 个月（范围，6-176），9 例患者在无治疗的情况下仍存活，2 例死亡。5 年无进展生存率和总生存率分别为 77.1%（95%可信区间：34.5-93.9）和 74.1%（95%可信区间：28.1-93.0）。
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