Maruzzo Marco, Martin-Liberal Juan, Messiou Christina, Miah Aisha, Thway Khin, Alvarado Rolyn, Judson Ian, Benson Charlotte
Sarcoma Unit, The Royal Marsden NHS Foundation Trust, Fulham Road, SW3 6JJ London, UK.
Radiology Department, The Royal Marsden NHS Foundation Trust, London, UK.
Clin Sarcoma Res. 2015 Feb 2;5:5. doi: 10.1186/s13569-015-0022-2. eCollection 2015.
Solitary Fibrous Tumour (SFT) is a rare soft tissue neoplasm, described in several locations in the body. It is classified as intermediate malignant potential with low risk of metastasis and has a low tendency to recur after primary surgery.
We performed a prospective data collection of the patients with SFT presented to the Royal Marsden Hospital from January to December 2013, and treated with pazopanib in first line. Demographics, anatomic primary sites, treatment and survival outcomes were collected from patients' electronic records.
13 patients (54% females) were identified with a median age of 51 years (range 37-77). Most of the patients (77%) were diagnosed with extra-thoracic SFT. All the patients received first line treatment with pazopanib for metastatic disease. Median overall survival (OS) was 13.3 months. Median progression free survival (PFS) was 4.7 months. No statistically significant difference was found in OS and PFS between primary thoracic SFT and primary extra-thoracic SFT. According to RECIST, one partial response (9%) and eight disease stabilizations (73%) were found as best responses. Using Choi criteria, there were 5 partial responses (46%) and 4 stabilizations (36%).
Our prospective data confirm that anti-angiogenic drugs are active in SFT. PFS and overall response do not appear significantly lower than other reported series on the same disease. Furthermore, pazopanib is a drug already licensed in soft tissue sarcomas and these data suggest its activity also in this particular subtype of sarcomas.
孤立性纤维性肿瘤(SFT)是一种罕见的软组织肿瘤,可发生于身体的多个部位。它被归类为具有低转移风险的中间恶性潜能肿瘤,初次手术后复发倾向较低。
我们对2013年1月至12月在皇家马斯登医院就诊并接受一线帕唑帕尼治疗的SFT患者进行了前瞻性数据收集。从患者的电子记录中收集人口统计学信息、解剖学原发部位、治疗和生存结果。
共确定13例患者(54%为女性),中位年龄51岁(范围37 - 77岁)。大多数患者(77%)被诊断为胸外SFT。所有患者均接受一线帕唑帕尼治疗转移性疾病。中位总生存期(OS)为13.3个月。中位无进展生存期(PFS)为4.7个月。原发性胸内SFT和原发性胸外SFT之间在OS和PFS方面未发现统计学显著差异。根据RECIST标准,最佳反应为1例部分缓解(9%)和8例病情稳定(73%)。根据Choi标准,有5例部分缓解(46%)和4例病情稳定(36%)。
我们的前瞻性数据证实抗血管生成药物在SFT中具有活性。PFS和总体反应似乎并不显著低于关于同一疾病的其他报道系列。此外,帕唑帕尼是一种已获软组织肉瘤许可的药物,这些数据表明其在这种特定亚型的肉瘤中也具有活性。