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初诊时伴转移的眼眶肉瘤:儿童肿瘤学组软组织肉瘤委员会的报告。

Orbital sarcoma with metastases at diagnosis: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

机构信息

Division of Pediatrics, The University of Texas M.D. Anderson Cancer Center, Houston, Texas 77030, USA.

出版信息

Pediatr Blood Cancer. 2010 Jul 1;54(7):1045-7. doi: 10.1002/pbc.22434.

Abstract

We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I-III. Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2-6.9 years). All patients had bone marrow involvement, including six with normal complete blood count at diagnosis. Cerebrospinal fluid was normal in six patients. Three patients survived >5 years, including one with local recurrence. In conclusion, further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients.

摘要

我们回顾了 7 名被诊断为 IV 期/IV 组眼眶肉瘤且接受 IRSG 方案 I-III 治疗的患者的临床病理特征和治疗结果。3 名患者患有胚胎性横纹肌肉瘤(RMS),2 名患者分别患有肺泡 RMS 或未分类肉瘤。诊断时的中位年龄为 1.8 岁(范围 0.2-6.9 岁)。所有患者均有骨髓受累,包括 6 例诊断时全血细胞计数正常的患者。6 例患者的脑脊液正常。3 名患者存活时间超过 5 年,其中 1 例局部复发。总之,需要进一步研究以确定眼眶肉瘤患者是否需要进行骨髓和 CSF 检查。

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