Epithelioid sarcoma: The clinicopathological complexities of this rare soft tissue sarcoma
Epithelioid sarcoma: The clinicopathological complexities of this rare soft tissue sarcoma
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DOI：
10.1007/bf02523657
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发表时间：
2000-04-01
影响因子：
3.7
通讯作者：
Fisher, C
中科院分区：
文献类型：
--
作者：
Spillane, AJ;Thomas, JM;Fisher, C
背景：上皮样肉瘤是一种罕见的高度恶性软组织肉瘤，有局部复发的倾向。文献局限于其他特征，如多灶性疾病的频率在介绍，提出的关系，主要病变的大小，预后，以及目前的分期系统，以预测预后的能力。方法：审查了皇家马斯登国民卫生服务信托基金（RMH）的经验，37例超过21年。结果：平均年龄为29岁，男性为主（2.7：1），远侧肢体最常见（56%）。5例患者出现多灶性局部疾病。19名仍然存活的患者的中位随访时间为88个月。5年和10年精算总生存率分别为70%和42%。肿瘤深到包埋筋膜有一个更差的预后比浅表肿瘤。区域转移事件也与总体生存率显著降低相关。局部复发、肿瘤大小≥ 5 cm和区域转移事件预测无远处转移生存率较差。肿瘤大小（5 cm vs.大于或等于5 cm）、局部复发事件、性别和部位不是生存率的显著预测因素。美国癌症联合委员会/国际抗癌联盟分期系统和最近提出的RMN分期系统的皇家马斯登国家卫生服务信托提供了上皮样肉瘤的预后分化差。5年精算局部复发率为35%。5年区域淋巴结转移率为23%。5年远处转移率为40%，以胸膜肺转移多见，胸膜肺转移伴胸腔积液者占35%。远处转移后中位生存期为8个月。结论：上皮样肉瘤与其他高级别软组织肉瘤相比具有不寻常的临床表现。它有多灶性疾病的倾向，局部复发，区域转移，特别是区域或远处转移性疾病后预后差。根据美国癌症联合委员会/国际抗癌联盟的大小和阶段是不可靠的预后预测因素。
Background: Epithelioid sarcoma is a rare high grade soft tissue sarcoma with a known propensity for locoregional recurrence. The literature is limited on other characteristics such as frequency of multifocal disease at presentation, the relationship of presenting size of the primary lesion to prognosis, and the ability of current staging systems to predict prognosis.Methods: Review of the Royal Marsden National Health Service Trust (RMH) experience of 37 cases over 21 years.Results: The mean age was 29 years, with male predominance (2.7:1), and distal limb locations were most common (56%). Five patients presented with multifocal local disease. Median follow-up was 88 months in the 19 patients still alive. The 5- and 10-year actuarial overall survival was 70% and 42%, respectively. Tumors deep to the investing fascia had a worse prognosis than superficial tumors. Regional metastasis events were also associated with significantly worse overall survival. Local recurrence, size of 5 cm or larger, and regional metastasis events were predictive of worse distant metastasis-free survival. Tumor size (5 cm vs. greater than or equal to 5 cm), local recurrence events, sex, and site were not significant predictors of survival. The American Joint Committee on Cancer/international Union Against Cancer staging systems and the recently proposed RMN staging system of the Royal Marsden National Health Service Trust provided poor differentiation of prognosis in epithelioid sarcoma. The 5-year actuarial local recurrence rate was 35%. The 5-year actuarial regional nodal metastasis rate was 23%. The actuarial 5-year distant metastasis rate was 40%, with pleuropulmonary metastases the must common sits of metastatic disease, and 35% of plcuropulmonary metastases presented with pleural effusion. Median post-distant metastasis survival was 8 months.Conclusions: Epithelioid sarcoma has unusual clinical behavior compared with other high grade soft tissue sarcoma. It has a propensity for multifocal disease at presentation, local recurrence, regional metastasis, and particularly poor prognosis after regional or distant metastatic disease. Size and stage according to the American Joint Committee on Cancer/international Union Against Cancer are unreliable predictors of prognosis.