Division of Pediatrics, The University of Texas M.D. Anderson Cancer Center, Houston, Texas 77030, USA.
Pediatr Blood Cancer. 2010 Jul 1;54(7):1045-7. doi: 10.1002/pbc.22434.
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.
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。
