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首页> 外文期刊>Pediatric blood & cancer >Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group.
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Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group.

机译：1978-1997年小组间横纹肌肉瘤研究组（IRSG）方案II-IV治疗的颅脑膜旁肉瘤和转移瘤（第4阶段）患者的结果：儿童肿瘤学组的报告。

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PURPOSE: Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV. PATIENTS: We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail. RESULTS: The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal-pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues. OUTCOME: The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06). CONCLUSIONS: Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynxasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease.
机译：目的：确定组间横纹肌肉瘤研究组（IRSG）方案II-IV治疗的颅脑膜旁肉瘤和并发转移的患者的预后。患者：我们在数据库中确定了91例患者，其中包括<21岁的新诊断为横纹肌肉瘤（RMS）和未分化肉瘤的受试者，并详细检查了他们的图表。结果：54例男性和37例女性的诊断时间<1-19岁。主要部位是鼻咽-鼻腔，中耳/乳突和咽旁区域（“较好”部位，占55％），鼻旁窦和颞下翼ery肌区域（“较差”部位，占42％）和其他部位（3％）。 68％的信息丰富的患者直接颅内扩张。诊断时主要的转移部位是肺（63％），骨髓（33％）和骨（27％）。治疗包括长春新碱，放线菌素D和环磷酰胺（VAC）化疗以及对原发肿瘤和多达五个转移部位/组织的放疗。结果：估计的10年无失败生存率（FFS）为32％（95％置信区间[CI]：22％，42％）。 60例患者进行性疾病（N = 49）或首次死亡（N = 11）;另一个发展为骨髓增生异常综合症并死亡。初次进展/复发的部位较远（55％），局部（12％），中枢神经系统扩张（8％），混合（6％）和不确定（18％）。表明10年FFS可能性的因素包括肿瘤发生在“更好”与“更差”部位（FFS 46％vs. 18％，P = 0.02）以及胚胎与其他组织学（FFS 37％vs. 19％，P = 0.06） ）。结论：某些转移性颅脑膜旁肉瘤患者可以治愈。前景最佳的患者的胚胎RMS位于鼻咽/鼻腔，中耳/乳突或咽旁区域。远处转移是最常见的复发类型，表明需要更有效的全身性药物来消除残留疾病。

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