Clinical Outcomes for Chest Wall Ewing Sarcoma: A Multi-Center Single Institution Experience

Abstract

We report tumor and treatment characteristics, oncologic outcomes, and treatment-associated toxicities in a cohort of chest wall Ewing sarcoma (cwES) patients treated at a single tertiary care institution. After IRB approval, patients with cwES treated from 1997-2022 were retrospectively reviewed. Patient, tumor, treatment, outcomes, and toxicity data were abstracted. Local control (LC), progression-free survival (PFS), and overall survival (OS) were defined from end of treatment and assessed using the Kaplan-Meier method. Log-rank test and unadjusted Cox models were performed to determine factors associated with outcomes. The cohort includes 45 patients. Median age at diagnosis was 19.8 years (range: 3.5 - 57.8 years). Five patients (11.1%) presented with pleural effusion and eight patients with lung metastases (17.8%). Two (4.4%) patients had metastatic disease outside the thorax. Median tumor volume (TV) was 138.6 mL (range: 3.0-6762.0 mL). All patients received VDC/IE chemotherapy. LC modality was surgery (S) in 21 patients (47%), radiation therapy (RT) in 5 (11%), and S+RT in 19 (42%). Median TV was larger in S+RT patients (319.4 mL, range: 5.3-6761.9 mL) compared to RT (152.3 mL, range: 20.4-366.9 mL) or S (70.4 mL, range: 3.1-1037.8 mL) (p = 0.03). R0 and R1 resections were performed in 36 (90%) and 4 (10%) patients, respectively. Proton beam therapy was used in 15 (63%) patients. Median dose was 50.40 Gy (range: 34.2 - 60 Gy) in 28 fractions to the primary tumor or post operative bed. Median dose for hemithorax (1 patient, 2.2%) and whole lung irradiation (7 patients, 15.6%) was 15.0 Gy (range: 15.0-15.0 Gy) in 10 fractions. Median follow-up was 2.38 years (range: 0 - 21.90 years). Five-year LC, PFS, and OS for all patients was 77.9% (95% CI, 65.3 - 92.9%), 54.2% (95% CI, 39.9 - 73.5%), and 63.5% (95% CI, 49.3 - 81.8%), respectively. In patients with localized disease, 5-year LC, PFS, and OS were 82.4% (95% CI, 67.9-99.8%), 66.4% (95% CI, 49.7-88.8%), and 71.3% (95% CI, 54.2-93.9%), respectively. Two-year LC by modality was 100% for RT (95% CI, 100-100%), 84.2% (95% CI, 69.3- 100%) for S and 73.3% (95% CI, 54 - 99.5%) for S+RT (p = 0.51). On univariate analysis, TV ≥ 200 mL was associated with a significantly worse 5-year OS (49.5%, TV ≥ 200 mL vs. 80.8%, TV < 200 mL; HR 4.44, p = 0.032) and PFS (35.2%, TV ≥ 200 mL vs. 76%, TV < 200 mL; HR 3.55, p = 0.025). TV ≥ 200 mL trended towards worse 5-year LC: 69.2% for TV ≥ 200 mL versus 81.5% for TV <200 mL [HR 2.26(95% CI 0.49 - 10.47), p = 0.287]. Overall, low rates of grade ≥2 toxicity were observed: 4 (8.9%) fatigue, 4 (8.9%) radiation dermatitis, 1 (2.2%) chyle leak, 3 (6.6%) scoliosis, 4 (8.9%) infection, 1 (2.2%) pneumonia, and 1 (2.2%) chest wall deformity. RT is a safe, effective local therapy for small to moderate cwES tumors. Patients with TV ≥ 200 mL had significantly worse survival outcomes and an inferior LC rate. This suggests large cwES tumors may benefit from an aggressive multi-modality approach.