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Outcome of Wilms tumor patients with bone metastasis enrolled on National Wilms Tumor Studies 1‐5: A report from the Children's Oncology Group

机译:Wilms肿瘤患者的骨转移患者患者纳入国家威尔斯肿瘤研究1-5:儿童肿瘤学院的报告

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Abstract Background Wilms tumor (WT) is the most common renal tumor in children. We describe the outcomes for patients with WT that metastasized to bone (WTBM) to assist in decision making for these uncommon patients. Procedure We retrospectively reviewed the research records of patients identified with WTBM from the National Wilms Tumor Study (NWTS 1–5) database. We then related overall survival (OS) to histology, chemotherapy, radiation therapy to bone, location of metastasis, and when bone metastasis presented. Results Thirty‐eight of 8609 patients enrolled on NWTS 1‐5 (0.44%) developed bone metastasis. Bone metastasis most commonly first occurred at progression or relapse (29/38, 76%). Five of thirty‐eight survived (13%) with the 5‐year OS following presentation of bone metastasis of 14.3% (95% CI: 2.7–25.8%). The primary cause of death was tumor (29/33, 88%). Of those who died, the median survival time was 10.9 months (range 0.49–61.4). Four of nine (44%) patients presenting at diagnosis and 3% (1/29) of patients presenting in progression or relapse survived ( P ?=?0.0075). Nineteen percent (5/26) of patients with favorable histology and 0% (0/12) with anaplastic histology survived ( P ?=?0.16). Of the five survivors, median follow‐up was 14 years (range 6.7–23.8). Radiation to metastatic bone sites was recorded in three of five survivors. No consistent chemotherapeutic approach appeared to be associated with disease outcome. Conclusion Bone metastasis is rare in patients with WT, occurring more commonly in progression or relapse than at initial diagnosis. Patients with WTBM have poor prognosis. We could not identify a consistent chemotherapeutic strategy associated with survival.
机译:摘要背景WILMS肿瘤(WT)是儿童中最常见的肾脏肿瘤。我们描述了WT患者的结果,该患者转移到骨骼(WTBM)以协助这些罕见患者的决策。程序我们回顾性地审查了来自国家Wilms肿瘤研究(NWTS 1-5)数据库的WTBM鉴定的患者的研究记录。然后我们将整体存活(OS)与组织学,化疗,放射疗法相关,转移的位置,以及骨转移呈现。结果386例8609例患者注册了NWT 1-5(0.44%)发育骨转移。骨转移最常见于进展或复发时(29/38,76%)。在介绍14.3%(95%CI:2.7-25.8%)后,38人幸存(13%)幸存下来(13%),5年的OS(95%:2.7-25.8%)。死亡原因是肿瘤(29/33,88%)。那些死亡的人,中位生存时间为10.9个月(范围0.49-61.4)。九(44%)患者中的四种(44%)患者患者患有进展或复发的患者的3%(1/29)存活(p?= 0.0075)。 19%(5/26)患者有利的组织学和0%(0/12),具有促进组织学幸存下来(P?= 0.16)。在五个幸存者中,中位随访14年(范围为6.7-23.8)。在五个幸存者中有三个记录转移性骨头位点的辐射。没有一致的化学治疗方法似乎与疾病结果相关。结论WT患者含有罕见的骨转移,比在初步诊断下发生的更常见于进展或复发。 WTBM患者预后差。我们无法识别与生存相关的一致化学治疗策略。

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