Adult rhabdomyosarcoma - Outcome following multimodality treatment
Adult rhabdomyosarcoma - Outcome following multimodality treatment
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DOI：
10.1002/cncr.10669
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发表时间：
2002-07-15
期刊：
影响因子：
6.2
通讯作者：
Benjamin, RS
中科院分区：
文献类型：
--
作者：
Little, DJ;Ballo, MT;Benjamin, RS
背景资料。儿童横纹肌肉瘤(RMS)预后较好。方法：回顾1960-1998年间收治的82例成人局部RMS患者的临床病理特征、治疗方法和疾病转归。排除诊断时有远处转移的患者。结果：患者年龄从17岁到84岁不等(中位数27岁)。组织学亚型为胚胎型(34%)、多形型(43%)和肺泡型(23%)。解剖起源部位为头颈部(52%)、躯干(26%)和四肢(7%)。51%的患者肿瘤大小在5厘米或更小。33%的患者出现区域淋巴转移。治疗包括单纯放疗占11%，放疗和手术占18%，放疗和化疗占34%，所有三种方式均占37%。中位随访期10.5年，10年无病生存率和总生存率分别为41%和40%。10年局部控制率、淋巴结控制率和转移控制率分别为75%、82%和53%。转移控制和生存的主要决定因素是原发肿瘤的大小(小于或等于5厘米对5厘米)。对于脑膜旁以外的部位，局部控制令人满意(10年成功率为87%)(10年时为50%)。对化疗有反应的患者的无转移期(10年时为72%)明显好于疾病无效者(10年时为19%)。结论：成人RMS是一种高度恶性的肿瘤，有显著的转移复发发生率。继续研究新的、可能更有效的化疗是至关重要的。局部控制对于脑膜旁以外的地方是令人满意的，在这些地方，可能需要新的放射技术，如强度调节治疗，以安全地提供足够的剂量。(C)2002年美国癌症协会。
BACKGROUND. Childhood rhabdomyosarcoma (RMS) has a relatively good prognosis. Outcome for adults with this disease is poorly documented due to its rarity.METHODS. The clinicopathologic features, treatment methods, and disease outcome were reviewed retrospectively for 82 adults with locoregional RMS treated between 1960 and 1998. Patients with distant metastasis at diagnosis were excluded. Actuarial univariate and multivariate statistical methods were used to evaluate outcome.RESULTS. Patient ages ranged from 17 to 84 years (median, 27 years). Histologic subtypes were embryonal (34%), pleomorphic (43%), and alveolar (23%). Anatomic sites of origin were head and neck (52%), trunk (26%), and extremity (7%). Tumor size was 5 cm or smaller in 51% of patients. Regional lymph node metastasis was present in 33% of patients at presentation. Treatment consisted of radiation alone in 11%, radiation and surgery in 18%, radiation and chemotherapy in 34%, and all three modalities in 37%. With a median follow-up of 10.5 years, the 10-year actuarial disease-free and overall survival rates were 41% and 40%, respectively. The 10-year actuarial local, lymph node, and metastatic control rates were 75%, 82%, and 53%, respectively. The major determinant of metastatic control and survival was primary tumor size (less than or equal to 5 vs. > 5 cm). Local control was satisfactory (10-year rate of 87%) for sites other than parameningeal (50% at 10 years). Patients whose disease responded to chemotherapy had a significantly better metastasis free period (72% at 10 years) than those whose disease failed to respond (19% at 10 years).CONCLUSIONS. Adult RMS is a highly malignant tumor with a significant incidence of metastatic recurrence. Continuing investigation of new and potentially more effective chemotherapy is crucial. Local control is satisfactory for sites other than parameningeal where new radiation technologies such as intensity-modulated therapy may be necessary to safely deliver adequate doses. (C) 2002 American Cancer Society.