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首页> 外文期刊>Journal of neuro-oncology. >Childhood pineoblastoma: experiences from the prospective multicenter trials HIT-SKK87, HIT-SKK92 and HIT91.
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Childhood pineoblastoma: experiences from the prospective multicenter trials HIT-SKK87, HIT-SKK92 and HIT91.

机译：儿童成纤维细胞瘤：前瞻性多中心试验HIT-SKK87，HIT-SKK92和HIT91的经验。

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OBJECTIVE: To analyze the outcome of children with pineoblastoma (PB), treated within the prospective multicenter trials HIT-SKK87, HIT-SKK92 and HIT91 of German-speaking countries. PATIENTS: We report on 11 children suffering from PB. Five children younger than 3 years of age received chemotherapy after surgery until eligible for radiotherapy (HIT-SKK87 and HIT-SKK92). Five of six children older than 3 years were treated after surgery with immediate chemotherapy and craniospinal irradiation, and one child received maintenance chemotherapy after postoperative radiotherapy (HIT91). RESULTS: Five of the six older children are still alive in continuous complete remission (CCR) with a median overall survival (OS) and progression free survival (PFS) of 7.9 years. Five of these six HIT91 patients responded to postoperative chemotherapy and radiotherapy. The only patient with tumor progression during initial chemotherapy achieved complete remission with radiotherapy and is alive. In contrast, all five young children died of tumor progression after a median OS of 0.9 years (PFS 0.6 years). They had either metastatic disease (M1) and/or postoperative residual tumor. Response to postoperative chemotherapy was lower than in the older age group, and only one of these children received radiotherapy. CONCLUSIONS: Combined chemotherapy and radiotherapy were feasible and effective in the older age group, leading to prolonged remissions in five of six children. Tumor biology may be more aggressive in younger children with PB, who presented more frequently with high-risk features at diagnosis and had poorer response rates to neoadjuvant postoperative chemotherapy. More intensified treatment regimens may be needed for young children with PB.
机译：目的：分析在德语国家的前瞻性多中心试验HIT-SKK87，HIT-SKK92和HIT91中治疗的成纤维细胞瘤（PB）儿童的结局。患者：我们报告了11名患有PB的儿童。 5名3岁以下的儿童在手术后接受了化疗，直到有资格接受放射治疗（HIT-SKK87和HIT-SKK92）。术后3岁以上的6名儿童中有5名在手术后接受了立即化疗和颅骨脊髓照射治疗，一名儿童在术后放疗（HIT91）后接受了维持化疗。结果：六个年龄较大的儿童中有五个仍在持续完全缓解（CCR）中存活，中位总生存期（OS）和无进展生存期（PFS）为7.9年。这六名HIT91患者中有五名对术后化疗和放疗有反应。仅有的在初始化疗期间肿瘤进展的患者通过放疗获得了完全缓解，并且还活着。相反，所有五个幼儿在中位OS为0.9年（PFS为0.6年）后死于肿瘤进展。他们患有转移性疾病（M1）和/或术后残留肿瘤。术后化学疗法的反应低于较大年龄组，这些儿童中只有一名接受了放疗。结论：联合化疗和放疗在较大年龄组中是可行和有效的，导致6名儿童中有5名儿童的缓解时间延长。较年轻的PB患儿的肿瘤生物学可能更具侵略性，这些患儿在诊断时表现出高风险特征，并且对新辅助术后化疗的反应率较差。对于PB的幼儿，可能需要更多的强化治疗方案。

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