Lex Johnathan R, Aoude Ahmed, Stevenson Jonathan D, Wunder Jay S, Evans Scott, Ferguson Peter C, Stavropoulos Nikolaos A, Jeys Lee, Goulding Krista, Turcotte Robert E
University of Birmingham, Birmingham B15 2TT, UK.
McGill University Health Centre, Montreal, QC, Canada H4A 3J1.
Sarcoma. 2018 Apr 1;2018:5392785. doi: 10.1155/2018/5392785. eCollection 2018.
Developing multiple soft tissue sarcomas (STSs) is a rare process, sparsely reported in the literature to date. Little is known about the pattern of disease development or outcomes in these patients. Patients were identified from three tertiary orthopaedic oncology centres in Canada and the UK. Patients who developed multiple extremity STSs were collated retrospectively from prospective oncology databases. A literature review using MEDLINE was also performed. Six patients were identified in the case series from these three institutions, and five studies were identified from the literature review. Overall, 17 patients were identified with a median age of 51 years (range: 19 to 77). The prevalence of this manifestation in STS patients is 1 in 1225. The median disease-free interval between diagnoses was 2.3 years (range: 0 to 19 years). Most patients developed the secondary STS in a metachronous pattern, the remaining, synchronously. The median survival after the first sarcoma was 6 years, and it was 1.6 years after the second sarcoma. The 5-year overall survival rate was 83.3% and 50% following the first and second STS diagnoses, respectively. A diagnosis of two STSs does not confer a worse prognosis than the diagnosis of a single STS. Developing a second STS is a rare event with no identifiable histological pattern of occurrence. Presentation in a metachronous pattern is more common. A high degree of vigilance is required in patients with a previous STS both to detect both local recurrence and to identify new masses remote from the previous STS site. Acquiring an early histological diagnosis should be attempted.
发生多发性软组织肉瘤(STS)是一个罕见的过程,迄今为止文献报道较少。对于这些患者疾病发展模式或预后情况知之甚少。从加拿大和英国的三个三级骨科肿瘤中心识别出患者。从前瞻性肿瘤数据库中回顾性整理出发生多发性肢体STS的患者。还使用MEDLINE进行了文献综述。在这三个机构的病例系列中识别出6例患者,在文献综述中识别出5项研究。总体而言,共识别出17例患者,中位年龄为51岁(范围:19至77岁)。这种表现形式在STS患者中的患病率为1/1225。两次诊断之间的无病间隔时间中位数为2.3年(范围:0至19年)。大多数患者的继发性STS以异时性模式发生,其余为同时性。首次肉瘤后的中位生存期为6年,第二次肉瘤后为1.6年。第一次和第二次STS诊断后的5年总生存率分别为83.3%和50%。诊断为两个STS并不比诊断为单个STS的预后更差。发生第二个STS是一种罕见事件,没有可识别的组织学发生模式。以异时性模式出现更为常见。对于既往有STS的患者,需要高度警惕,既要检测局部复发,也要识别远离既往STS部位的新肿块。应尝试尽早获得组织学诊断。