RHABDOMYOSARCOMA OF THE NOSE AND PARANASAL SINUSES IN ADULTS AND CHILDREN
RHABDOMYOSARCOMA OF THE NOSE AND PARANASAL SINUSES IN ADULTS AND CHILDREN
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DOI：
10.1016/s0194-5998(95)70246-6
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发表时间：
1995-02-01
影响因子：
3.4
通讯作者：
ELNAGGAR, A
中科院分区：
文献类型：
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作者：
CALLENDER, TA;WEBER, RS;ELNAGGAR, A
目的：探讨鼻及副鼻窦横纹肌肉瘤儿童和成人的生物学行为、复发危险因素以及多模式治疗对预后的影响。设计：对医疗记录进行回顾性研究。患者：在36年的时间里，37例经组织学证实的鼻和鼻窦横纹肌肉瘤患者（中位年龄23.0岁）接受了治疗，中位随访时间为28个月（范围1至260个月）。组织学亚型包括胚胎亚型，16个；肺泡,15;混合,1;非机密的，5个。38%存在宫颈转移。手术治疗4例；放射治疗,3;化疗,3;手术与放疗1；手术与化疗，1；化疗和放疗，24人；化疗，放疗和手术，1。结果：总5年生存率为44%。对于化疗+放疗或化疗+放疗+手术的患者，5年生存率为60%，而接受其他形式治疗的患者为19%。与较差生存率相关的因素是成年发病、肺泡组织学和接受全身化疗少于1年。化疗时间超过1年的患者5年生存率为82%，而化疗时间少于1年的患者5年生存率为71%。生存率的提高与远处转移的发生率降低有关。结论：化疗和放疗联合治疗可能是鼻窦和副鼻窦横纹肌肉瘤局部控制的最佳方法。发生局部病变的危险性高，除原发部位外，需要对颈部进行全面放疗。对于化疗和放疗后残留病变的患者，应保留手术切除。化疗1年以上与生存率提高相关，因为转移性疾病的发生率降低。
OBJECTIVE: To determine the biologic behavior, risk factors for recurrence, and influence of multimodality therapy on outcome for children and adults with rhabdomyosarcoma of the nose and paranasal sinuses.DESIGN: Retrospective review of medical records.PATIENTS: During a 36-year period 37 patients (median age, 23.0 years) with histologically confirmed rhabdomyosarcoma of the nose and paranasal sinuses were treated for cure, Median follow-up was 28 months (range, 1 to 260 months). Histologic subtypes included embryonal, 16; alveolar, 15; mixed, 1; and unclassified, 5. Cervical metastases were present in 38%. Patients were treated with surgery, 4; radiotherapy, 3; chemotherapy, 3; surgery and radiotherapy 1; surgery and chemotherapy, 1; chemotherapy and radiotherapy, 24; and chemotherapy, radiotherapy, and surgery, 1.RESULTS: The overall 5-year survival was 44%. For patients treated with chemotherapy and radiotherapy or chemotherapy, radiotherapy, and surgery, the 5-year survival was 60%, compared with 19% for patients treated with the other forms of therapy. Factors associated with poorer survival were adult onset of disease, alveolar histology, and treatment with systemic chemotherapy for less than 1 year. Patients receiving chemotherapy for greater than 1 year had a 5-year survival of 82%, compared with 71% for those with less than 1 year of treatment. Improved survival was associated with a lower incidence of distant metastasis.CONCLUSIONS: A combination of chemotherapy and radiotherapy may provide Che best means of obtaining local-regional control for rhabdomyosarcoma arising in the nose and paranasal sinuses. The risk of regional disease is high, requiring comprehensive radiotherapy to the neck in addition Co the primary site. Surgical resection should be reserved for patients with residual disease after chemotherapy and radiotherapy. Administration of chemotherapy for more than 1 year is associated with improved survival because of a decreased incidence of metastatic disease.