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儿童和青少年横纹肌肉瘤诊断时的骨髓转移。来自横纹肌肉瘤协作组研究的报告。

Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. A report from the intergroup rhabdomyosarcoma study.

作者信息

Ruymann F B, Newton W A, Ragab A H, Donaldson M H, Foulkes M

出版信息

Cancer. 1984 Jan 15;53(2):368-73. doi: 10.1002/1097-0142(19840115)53:2<368::aid-cncr2820530233>3.0.co;2-3.

DOI:10.1002/1097-0142(19840115)53:2<368::aid-cncr2820530233>3.0.co;2-3
PMID:6546301
Abstract

Thirty children and adolescents with rhabdomyosarcoma (RMS) entered on IRS-1 in a 5-year period had bone marrow metastases (BMM) at diagnosis. BMM at diagnosis occurred in 30/103 patients (29%) with Clinical Group IV RMS. On the average, patients with BMM were older with more extremity primaries than other IRS-1 patients. Alveolar histology was present in 15/30 patients (50%) with BMM, compared to 19% on IRS-1. Eleven patients received vincristine, dactinomycin, cyclophosphamide (VAC), while 19 patients received VAC with Adriamycin (ADR). The overall complete response (CR) rate was 60%. The median disease-free survival times by treatment were 17 weeks with VAC and 32 weeks for VAC + ADR (P greater than 0.10). Relapse from CR in Clinical Group IV patients with BMM occurred in 16/18 patients (89%) and was significantly higher than in 17/29 Clinical Group IV patients (59%) without BMM (P less than 0.035). BMM at diagnosis in children and adolescents with RMS is frequently associated with alveolar histology and is a poor prognostic sign.

摘要

在5年期间进入IRS-1研究的30例横纹肌肉瘤(RMS)儿童和青少年在诊断时发生了骨髓转移(BMM)。在临床IV组RMS的103例患者中,有30例(29%)在诊断时出现BMM。平均而言,发生BMM的患者比其他IRS-1患者年龄更大,肢体原发肿瘤更多。30例发生BMM的患者中有15例(50%)为肺泡组织学类型,而在IRS-1研究中该比例为19%。11例患者接受长春新碱、放线菌素D、环磷酰胺(VAC)治疗,19例患者接受VAC联合阿霉素(ADR)治疗。总体完全缓解(CR)率为60%。按治疗方法计算,无病生存时间的中位数VAC组为17周,VAC + ADR组为32周(P大于0.10)。临床IV组发生BMM的患者中,18例有16例(89%)CR后复发,显著高于临床IV组中未发生BMM的29例患者中的17例(59%)(P小于0.035)。RMS儿童和青少年诊断时出现BMM常与肺泡组织学类型相关,是预后不良的征象。

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