Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts.
Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts.
Mod Pathol. 2023 Mar;36(3):100069. doi: 10.1016/j.modpat.2022.100069. Epub 2023 Jan 10.
Dedifferentiated chondrosarcoma is rare, aggressive, and microscopically bimorphic. How pathologic features such as the amounts of dedifferentiation affect prognosis remains unclear. We evaluated the percentages and sizes of dedifferentiation in a consecutive institutional series of dedifferentiated chondrosarcomas from 1999 to 2021. The statistical analysis included cox proportional hazard models and log-rank tests. Of the 67 patients (26 women, 41 men; age, 39 to >89 [median 61] years; 2 with Ollier disease), 58 presented de novo; 9 were identified with conventional chondrosarcomas 0.6-13.2 years (median, 5.5 years) prior. Pathologic fracture and distant metastases were noted in 27 and 7 patients at presentation. The tumors involved the femur (n = 27), pelvis (n = 22), humerus (n = 7), tibia (n = 4), scapula/ribs (n = 4), spine (n = 2), and clivus (n = 1). In the 56 resections, the tumors ranged in size from 3.5 to 46.0 cm (median, 11.5 cm) and contained 1%-99.5% (median, 70%) dedifferentiated components that ranged in size from 0.6 to 24.0 cm (median, 7.3 cm). No correlation was noted between total size and percentage of dedifferentiation. The dedifferentiated components were typically fibrosarcomatous or osteosarcomatous, whereas the associated cartilaginous components were predominantly grade 1-2, rarely enchondromas or grade 3. The entire cohort's median overall survival and progression-free survival were 11.8 and 5.4 months, respectively. In the resected cohort, although the total size was not prognostic, the percentage of dedifferentiation ≥20% and size of dedifferentiation >3.0 cm each predicted worse overall survival (9.9 vs 72.5 months; HR, 3.76; 95% CI, 1.27-11.14; P = .02; 8.7 vs 58.9 months; HR, 3.03; 95% CI, 1.21-7.57; P = .02, respectively) and progression-free survival (5.3 vs 62.1 months; HR, 3.05; 95% CI, 1.13-8.28; P = .03; 5.3 vs 56.6 months; HR, 2.50; 95% CI, 1.06-5.88; P = .04, respectively). In conclusion, both the percentages and sizes of dedifferentiation were better prognostic predictors than total tumor sizes in dedifferentiated chondrosarcomas, highlighting the utility of their pathologic evaluations.
去分化软骨肉瘤较为罕见，侵袭性强，显微镜下呈双相型。去分化程度等病理特征对预后的影响尚不清楚。我们评估了 1999 年至 2021 年连续机构系列去分化软骨肉瘤中去分化的百分比和大小。统计分析包括 Cox 比例风险模型和对数秩检验。在 67 例患者（26 名女性，41 名男性；年龄 39 岁至>89 岁[中位数 61 岁]；2 例患有 Ollier 病）中，58 例为初发病例；9 例在发病前 0.6-13.2 年（中位数 5.5 年）被诊断为常规软骨肉瘤。27 例患者在就诊时出现病理性骨折，7 例患者出现远处转移。肿瘤累及股骨（n=27）、骨盆（n=22）、肱骨（n=7）、胫骨（n=4）、肩胛骨/肋骨（n=4）、脊柱（n=2）和斜坡（n=1）。在 56 例切除标本中，肿瘤大小为 3.5-46.0cm（中位数 11.5cm），含有 1%-99.5%（中位数 70%）的去分化成分，大小为 0.6-24.0cm（中位数 7.3cm）。总大小与去分化百分比之间无相关性。去分化成分通常为纤维肉瘤样或骨肉瘤样，而相关的软骨成分主要为 1-2 级，很少为软骨瘤或 3 级。整个队列的中位总生存期和无进展生存期分别为 11.8 个月和 5.4 个月。在切除的队列中，尽管总大小没有预后意义，但去分化百分比≥20%和去分化大小>3.0cm 均预测总生存期较差（9.9 个月 vs 72.5 个月；HR，3.76；95%CI，1.27-11.14；P=0.02）和无进展生存期（5.3 个月 vs 62.1 个月；HR，3.05；95%CI，1.13-8.28；P=0.03）。总之，去分化软骨肉瘤的去分化百分比和大小均优于总肿瘤大小，是更好的预后预测指标，突出了其病理评估的效用。
