Callender T A, Weber R S, Janjan N, Benjamin R, Zaher M, Wolf P, el-Naggar A
Department of Head and Neck Surgery, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Otolaryngol Head Neck Surg. 1995 Feb;112(2):252-7. doi: 10.1016/S0194-59989570246-6.
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.
Retrospective review of medical records.
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.
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.
A combination of chemotherapy and radiotherapy may provide the 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 to 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.
确定鼻及鼻窦横纹肌肉瘤患儿及成人的生物学行为、复发危险因素以及多模式治疗对其预后的影响。
对病历进行回顾性分析。
在36年期间，37例经组织学确诊的鼻及鼻窦横纹肌肉瘤患者接受了根治性治疗。中位年龄23.0岁，中位随访时间28个月（范围1至260个月）。组织学亚型包括胚胎型16例、肺泡型15例、混合型1例、未分类5例。38%的患者有颈部转移。治疗方式包括手术4例、放疗3例、化疗3例、手术加放疗1例、手术加化疗1例、化疗加放疗24例、化疗、放疗加手术1例。
总体5年生存率为44%。接受化疗加放疗或化疗、放疗加手术的患者5年生存率为60%，而接受其他治疗方式的患者为19%。与较差生存率相关的因素包括成人发病、肺泡型组织学以及全身化疗时间少于1年。接受化疗超过1年的患者5年生存率为82%，而治疗时间少于1年的患者为71%。生存率提高与远处转移发生率降低有关。
化疗和放疗联合可能是获得鼻及鼻窦横纹肌肉瘤局部区域控制的最佳方法。区域疾病风险高，除原发部位外还需对颈部进行全面放疗。手术切除应保留给化疗和放疗后有残留疾病的患者。化疗超过1年与生存率提高相关，因为转移性疾病发生率降低。
