Di Masi Paola, Colangeli Marco, Simonetti Mario, Bianchi Giuseppe, Righi Alberto, Bilancia Gabriele, Palmerini Emanuela, Crombé Amandine, Spinnato Paolo
Diagnostic and Interventional Radiology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Department of Orthopaedic Oncology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Diagnostics (Basel). 2025 Apr 17;15(8):1027. doi: 10.3390/diagnostics15081027.
Clear cell sarcoma (CCS) is a very uncommon, aggressive soft-tissue sarcoma (STS) with a dismal prognosis. In the current literature, there are very limited data focused on the radiological features of CCS. Our study's objective was to describe CCS pre-treatment's peculiar imaging characteristics (MRI above all) and to assess if some radiologic features may predict patients' outcomes with regard to the occurrence of distant metastases. : Our single-center experience includes all the patients with a histological diagnosis of CCS and available radiological and clinical data: 14 patients (8M, 6F, mean age 39.4 years old) were included. The available pre-treatment MRI or contrast-enhanced computed tomography (CECT) studies were examined using an analytical grid that incorporated characteristics from the most recent STS research. The occurrence of metastatic disease was matched with radiological features from baseline imaging studies. : MRI was available in 13 patients and CECT in 1 patient. The mean longest diameter (LD) was 50.5 mm ± 29.2. In 10 cases (71.4%), the tumor was deeply seated. MRI revealed a slightly high signal intensity (SI) on T1-WI and a high SI on T2-WI in every subject. At baseline, metastases were already present in 5/14 (35.7%) patients, 3 more developed metastases during the following 5 years (8/14, 57.1%), and 2 additional developed late-onset metastasis after more than 5 years from the diagnosis (total of 10/14 metastatic patients 71.4%). LD and metastasis at diagnosis were significantly correlated (Pearson correlation = 72%, -value = 0.004). A pre-treatment LD > 4 cm was significantly associated with the development of distant metastases within 5 years from diagnosis and in subsequent follow-up ( = 0.0003). LD > 4 cm represents an increase in risk of metastatic disease within 5 years and during the course of follow-up (OR = 195.00, 95%CI: 3.36-11285.55, = 0.01). The presence of MRI signs of macroscopic necrosis represented an increase in risk of metastatic disease within 5 years (OR = 15.00, 95%CI: 1.03-218.31- = 0.04). The identification of MRI features of aggressive biology is a key element for an early referral to sarcoma centers. In our study, a LD > 4 cm and the presence of MRI signs of macroscopic necrosis at the baseline images resulted in being a predictor of metastatic disease.
透明细胞肉瘤（CCS）是一种非常罕见的侵袭性软组织肉瘤（STS），预后较差。在当前文献中，关于CCS放射学特征的数据非常有限。我们研究的目的是描述CCS治疗前独特的影像学特征（主要是MRI），并评估某些放射学特征是否可以预测患者发生远处转移的预后。：我们的单中心经验纳入了所有经组织学诊断为CCS且有可用放射学和临床数据的患者：共纳入14例患者（8例男性，6例女性，平均年龄39.4岁）。使用结合了最新STS研究特征的分析网格对可用的治疗前MRI或增强计算机断层扫描（CECT）研究进行检查。将转移性疾病的发生情况与基线影像学研究的放射学特征进行匹配。：13例患者有MRI数据，1例有CECT数据。平均最长径（LD）为50.5 mm±29.2。10例（71.4%）肿瘤位置较深。MRI显示所有患者在T1加权像（T1-WI）上呈稍高信号强度（SI），在T2加权像（T2-WI）上呈高SI。基线时，5/14（35.7%）患者已出现转移，在接下来的5年中有3例出现转移（8/14，57.1%），在诊断后5年以上又有2例出现迟发性转移（共10/14例转移患者，71.4%）。诊断时的LD与转移显著相关（Pearson相关系数=72%，P值=0.004）。治疗前LD>4 cm与诊断后5年内及后续随访中远处转移的发生显著相关（P=0.0003）。LD>4 cm表示5年内及随访期间发生转移性疾病的风险增加（比值比[OR]=195.00，95%置信区间[CI]：3.36-11285.55，P=0.01）。MRI显示宏观坏死的征象表示5年内发生转移性疾病的风险增加（OR=15.00，95%CI：1.03-218.31，P=0.04）。识别具有侵袭性生物学行为的MRI特征是早期转诊至肉瘤中心的关键因素。在我们的研究中，基线图像上LD>4 cm以及MRI显示宏观坏死的征象是转移性疾病的预测指标。
