Tigchelaar Seth S, Frey Christopher, Sivaraj Dharshan, Segovia Nicole A, Mohler David G, Steffner Robert J, Avedian Raffi S
Department of Orthopedic Surgery, Stanford University, Stanford, CA 94305, USA.
J Pers Med. 2022 Feb 24;12(3):345. doi: 10.3390/jpm12030345.
Leiomyosarcomas (LMS) are a heterogenous group of malignant mesenchymal neoplasms with smooth muscle origin and are classified as either non-uterine (NULMS) or uterine (ULMS). Metastatic pattern, prognostic factors, and ideal staging/surveillance studies for truncal and extremity LMS have not been defined. A retrospective analysis of patients diagnosed with histopathology-confirmed truncal or extremity LMS between 2009 and 2019 was conducted. Data collected included demographics, tumor characteristics, staging, surveillance, and survival endpoints. The primary site was defined as: (1) extremity, (2) flank/Pelvis, or (3) chest wall/Spine. We identified 73 patients, 23.3% of which had metastatic LMS at primary diagnosis, while 68.5% developed metastatic disease at any point. The mean metastatic-free survival from primary diagnosis of localized LMS was 3.0 ± 2.8 years. Analysis of prognostic factors revealed that greater age (≥50 years) at initial diagnosis (OR = 3.74, p = 0.0003), higher tumor differentiation scores (OR = 12.09, p = 0.002), and higher tumor necrosis scores (OR = 3.65, p = 0.026) were significantly associated with metastases. Older patients (≥50 years, OR = 4.76, p = 0.017), patients with larger tumors (≥5 cm or ≥10 cm, OR = 2.12, p = 0.02, OR = 1.92, p = 0.029, respectively), higher differentiation scores (OR = 15.92, p = 0.013), and higher necrosis scores (OR = 4.68, p = 0.044) show worse survival outcomes. Analysis of imaging modality during initial staging and during surveillance showed greater tumor detection frequency when PET imaging was employed, compared to CT imaging (p < 0.0001). In conclusion, truncal and peripheral extremity LMS is an aggressive tumor with high metastatic potential and mortality. While there is a significant risk of metastases to lungs, extra-pulmonary tumors are relatively frequent, and broad surveillance may be warranted.
平滑肌肉瘤(LMS)是一组异质性的恶性间叶性肿瘤,起源于平滑肌,分为非子宫型(NULMS)或子宫型(ULMS)。躯干和四肢LMS的转移模式、预后因素以及理想的分期/监测研究尚未明确。对2009年至2019年间经组织病理学确诊为躯干或四肢LMS的患者进行了回顾性分析。收集的数据包括人口统计学、肿瘤特征、分期、监测和生存终点。原发部位定义为:(1)四肢,(2)侧腹/骨盆,或(3)胸壁/脊柱。我们共纳入73例患者,其中23.3%在初诊时即有转移性LMS,而68.5%在任何时候都出现了转移性疾病。局限性LMS初诊后的平均无转移生存期为3.0±2.8年。预后因素分析显示,初诊时年龄较大(≥50岁)(OR = 3.74,p = 0.0003)、肿瘤分化评分较高(OR = 12.09,p = 0.002)以及肿瘤坏死评分较高(OR = 3.65,p = 0.026)与转移显著相关。年龄较大的患者(≥50岁,OR = 4.76,p = 0.017)、肿瘤较大的患者(≥5 cm或≥10 cm,OR分别为2.12,p = 0.02;OR = 1.92,p = 0.029)、分化评分较高(OR = 15.92,p = 0.013)以及坏死评分较高(OR = 4.68,p = 0.044)的患者生存结局较差。初始分期和监测期间影像学检查方式的分析显示,与CT成像相比,采用PET成像时肿瘤检测频率更高(p < 0.0001)。总之,躯干和外周四肢LMS是一种具有高转移潜能和死亡率的侵袭性肿瘤。虽然转移至肺部的风险很大,但肺外肿瘤相对常见,可能需要进行广泛的监测。