低级别软骨肉瘤(LG-CS),包括非典型软骨肿瘤(ACT),是局部侵袭性病变。讨论的重点在于良性病变或侵袭性软骨肿瘤之间的鉴别诊断及其治疗:病灶内刮治或广泛切除。本研究介绍了21例LG-CS的手术治疗结果。方法本回顾性研究包括2013年至2021年接受手术治疗的21例LG-CS患者。十四个位于附肢骨骼中,轴向有七个(肩胛骨,脊柱,或骨盆)。死亡率,复发,转移性疾病,总生存率,无复发生存率,分析了每种手术类型和每种疾病位置的无转移性疾病生存率。在进行切除的情况下,还记录了手术并发症和残留肿瘤。使用Kaplan-Meier方法计算存活率。结果13例患者行病灶内刮治(11例阑尾,2例轴性病灶),8例接受了广泛切除(5个轴向和3个阑尾)。随访期间有6次复发,43%的轴性病变复发,在轴向收缩的情况下上升到100%。阑尾性LG-CS在21%的病例中复发,只有18%的刮除的阑尾病变未根除。整个随访的总生存率为90.5%,5年生存率为83%(12例患者有足够的随访)。切除病例的无复发和无转移生存率较高,75%和87.5%,vs.刮治69.2%和76.9%,分别。在9%的案例中,术前活检与手术标本的病理不一致。讨论LG-CS和ACT被描述为具有高存活率和低转移性疾病的可能性。出于这个原因,这些病变的治疗理念会发生变化,以反映这些特征。病灶内刮治被认为是一种侵入性较小的技术,用于根除非典型软骨肿瘤,并且并发症越来越少,这与我们的发现是一致的。诊断,然而,是具有挑战性的;错误评级是经常发生的,应该加以考虑。由于对高级别病变治疗不足的风险,一些作者仍然认为广泛切除术是首选的治疗方法.我们观察到生存期更长的趋势,减少复发,和广泛切除的转移性疾病。转移性疾病高于预期,在19%的病例中,并且总是与局部复发有关。结论LG-CS仍然是诊断和治疗的挑战;患者选择是根本。总生存率高,独立于治疗选择或病变位置。我们发现转移性疾病的发生率高于文献中描述的;这,再加上9%的错误率,反映了术前诊断的困难和将高级别软骨肉瘤作为低度病变治疗的风险。应使用更大的样本进行更多的研究,以获得统计上可靠的结果。
Introduction Low-grade chondrosarcomas (LG-CS), including atypical cartilaginous tumors (ACT), are locally aggressive lesions. The focus of the discussion sits on the differential diagnosis between benign lesions or aggressive cartilaginous tumors and on their treatment: intralesional curettage or wide resection. This study presents the results obtained in the surgical treatment of 21 cases of LG-CS. Methods This retrospective study includes 21 consecutive patients from a single center with LG-CS who underwent surgery from 2013 to 2021. Fourteen were located in the appendicular skeleton, and seven in the axial (shoulder blade, spine, or pelvis). Mortality rate, recurrence, metastatic disease, overall survival, recurrence-free survival, and metastatic disease-free survival were analyzed for each type of procedure and each disease location. Operative complications and residual tumors were also recorded in cases where resection was performed. Survival was calculated using the Kaplan-Meier method. Results Thirteen patients underwent intralesional curettage (11 appendicular and 2 axial lesions), and eight underwent wide resection (5 axial and 3 appendicular). There were six recurrences during the follow-up, 43% of the axial lesions recurred, rising to 100% in axial curetted ones. Appendicular LG-CS recurred in 21% of cases, and only 18% of curetted appendicular lesions were not eradicated. The overall survival for the entire follow-up is 90.5%, and the 5-year survival rate is 83% (12 patients have adequate follow-up). Recurrence-free and metastasis-free survival were higher in resection cases, with 75% and 87.5%, vs. curettage 69.2% and 76.9%, respectively. In 9% of cases, the preoperative biopsy was inconsistent with the pathology of the surgical specimen. Discussion LG-CS and ACT are described as having high survival and low potential for metastatic disease. For this reason, these lesions are subject to a change in treatment philosophy to reflect these characteristics. Intra-lesional curettage is advocated as a less invasive technique for eradicating atypical cartilage tumors and has fewer and less severe complications, which was in accordance with our findings. Diagnosis, however, is challenging; misgrading is frequent and should be considered. Because of this risk of under-treating higher-grade lesions, some authors still defend wide-resection as the treatment of choice. We observed a trend towards longer survival, less recurrence, and metastatic disease with wide resection. Metastatic disease was higher than expected, present in 19% of cases, and always associated with local recurrence. Conclusion LG-CS is still a diagnostic and treatment challenge; patient selection is fundamental. Overall survival is high, independent of treatment choice or lesion location. We found a higher rate of metastatic disease than described in the literature; this, coupled with a misgrading rate of 9%, reflects the difficulty of preoperative diagnosis and the risk of treating high-grade chondrosarcomas as a low-grade lesion. More studies should be carried out with larger samples to obtain statistically robust results.