Alveolar soft part sarcoma: progress toward improvement in survival? A population-based study.
Alveolar soft part sarcoma: progress toward improvement in survival? A population-based study.
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牙槽软部分肉瘤：朝着改善生存的进展？一项基于人群的研究。
DOI：
10.1186/s12885-022-09968-5
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发表时间：
2022-08-15
期刊：
影响因子：
3.8
通讯作者：
中科院分区：
文献类型：
--
作者：
肺泡性软组织肉瘤(ASPS)是一种罕见的软组织肉瘤组织学亚型，对传统的细胞毒化疗仍然是无效的。我们的目的是描述ASPS的特征，并调查在过去十年中肿瘤学结果是否有所改善。对2006年至2017年从日本骨与软组织肿瘤登记处确诊的120例新诊断的自闭症患者进行了回顾性分析。研究队列包括34名(28%)局限性ASPS患者和86名(72%)确诊时有转移性疾病的患者。5年疾病特异性生存率(DSS)在所有患者中为68%，在局部和转移癌中分别为86%和62%(p = 0.019)。发病时转移是影响预后的唯一不良因素(危险比[HR]：7.65；p = 0.048)。 >； 25年(80%；p = 0.023)，有深层肿瘤(75%；p = 0.002)，肿瘤 >； 5 cm(5-10 cm，81%； >； 10 cm，81%；p <； 0.001)在出现时更有可能发生转移。在局限性ASP患者中，辅助化疗或放射治疗不影响生存率，13例(45%)患者发生肺(n = )12例(92%)和脑(n = 2)(15%)远处转移。在转移性骨肉瘤患者中(肺，n = 85[99%]；骨，n = 12[14%]；脑n = 9[11%])，原发灶或转移灶的手术并不影响存活率。接受帕佐帕尼治疗的患者存活时间延长(p = 0.045)，但接受阿霉素为基础的细胞毒化疗的患者存活时间没有延长。总体而言，自2012年帕佐帕尼被批准用于晚期疾病以来，转移性ASPS的DSS有所改善(5年DSS，从58%到65%)，尽管没有统计学上的显著差异(p = 0.117)。这项全国性的研究证实了ASPS的一个独特特征，即经常发生肺和脑转移，但临床病程缓慢。在引入靶向治疗后，延长生存期的总体趋势鼓励人们继续努力，为这种具有治疗耐药性的软组织肉瘤开发新的治疗方案。网上版载有补充材料，可在10.1186/s12885-022-09968-5查阅。
Alveolar soft part sarcoma (ASPS) is a rare histological subtype of soft-tissue sarcoma, which remains refractory to conventional cytotoxic chemotherapy. We aimed to characterize ASPS and investigate whether the oncological outcome has improved over the past decade. One hundred and twenty patients with newly diagnosed ASPS from 2006 to 2017, identified from the Bone and Soft-Tissue Tumor Registry in Japan, were analyzed retrospectively. The study cohort comprised 34 (28%) patients with localized ASPS and 86 (72%) with metastatic disease at presentation. The 5-year disease-specific survival (DSS) was 68% for all patients and 86% and 62% for localized and metastatic disease, respectively (p = 0.019). Metastasis at presentation was the only adverse prognostic factor for DSS (hazard ratio [HR]: 7.65; p = 0.048). Patients who were > 25 years (80%; p = 0.023), had deep-seated tumors (75%; p = 0.002), and tumors > 5 cm (5–10 cm, 81%; > 10 cm, 81%; p < 0.001) were more likely to have metastases at presentation. In patients with localized ASPS, adjuvant chemotherapy or radiotherapy did not affect survival, and 13 patients (45%) developed distant metastases in the lung (n = 12, 92%) and brain (n = 2, 15%). In patients with metastatic ASPS (lung, n = 85 [99%]; bone, n = 12 [14%]; and brain n = 9 [11%]), surgery for the primary or metastatic site did not affect survival. Prolonged survival was seen in patients who received pazopanib treatment (p = 0.045), but not in those who received doxorubicin-based cytotoxic chemotherapy. Overall, improved DSS for metastatic ASPS has been observed since 2012 (5-year DSS, from 58 to 65%) when pazopanib was approved for advanced diseases, although without a statistically significant difference (p = 0.117). The national study confirmed a unique feature of ASPS with frequent metastasis to the lung and brain but an indolent clinical course. An overall trend toward prolonged survival after the introduction of targeted therapy encourages continuous efforts to develop novel therapeutic options for this therapeutically resistant soft-tissue sarcoma. The online version contains supplementary material available at 10.1186/s12885-022-09968-5.
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