PQA 02 - Pediatric Cancer, Sarcoma and Cutaneous Tumors, Medical Education & Professional Development, and Health Services Research
Presenter(s)
O. Appana1, V. Yildiz2, J. Mayerson3, J. Alexander1, C. Quinion1, T. Scharschmidt4, M. Husain5, K. E. Haglund6, V. Grignol7, J. Beane1, H. G. Kim1, M. Xu5, D. Liebner8, D. J. Konieczkowski9, and G. Tinoco8; 1The Ohio State University, Columbus, OH, 2Center for Biostatistics, The Ohio State University Medical Center, Columbus, OH, 3The James Cancer Hospital and Solove Research Institute, The Ohio State University Comprehensive Cancer Center, Columbus, OH, 4Ohio State Univeristy Medical Center, Cleveland, OH, 5The Ohio State University Wexner Medical Center, Columbus, OH, 6Department of Radiation Oncology, The Ohio State University Wexner Medical Center, Columbus, OH, Columbus, OH, 7Ohio State, Columbus, OH, 8Department of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, OH, 9Department of Radiation Oncology, James Cancer Hospital/Wexner Medical Center, The Ohio State University, Columbus, OH
Characteristics, Management, and Outcomes of Localized Chondrosarcoma: A Single-Institution Retrospective Study
Purpose/Objective(s):
To describe clinical characteristics and treatment patterns of localized chondrosarcoma (CS) and identify factors associated with survival.
Materials/Methods:
We retrospectively reviewed localized CS patients evaluated at The Ohio State University between January 1, 2010 to March 7, 2022. Demographics, histologic subtypes, tumor characteristics, treatments factors, and survival outcomes including overall survival (OS), progression free survival (PFS), local recurrence free survival (LRFS), and distant metastasis free survival (DMFS) were analyzed.
Results:
Of 121 patients identified, 102 were evaluable with adequate follow-up. 92.1% were white and 51.0% were male. Median age at diagnosis was 58.5 (range: 18-85).
81.4% had Conventional CS, 15.7% Dedifferentiated CS, 2.0% Mesenchymal CS, and 1.0% Clear cell CS. At diagnosis, 47.1% of tumors were Grade I, 30.4% Grade II, and 22.6% Grade III. The median primary tumor size was 6.1 cm. The lower extremities were the most common primary site (30.4%), followed by the pelvis (20.6%), thoracoabdominal (20.6%), skull base/head and neck (15.7%), and upper extremities (12.8%).
99.0% of patients received surgery, of whom R0 margins were achieved in 71.7%, R1 margins in 14.1%, GTR (R0/R1 undefined) in 9.8%, and R2 margins in 4.3%. 16.7% of patients received systemic therapy, most frequently Pazopanib (58.8%). 21.6% of patients received radiation therapy, most frequently in the skull base region (50%). With a median follow-up among surviving patients of 43.1 months, 5-year OS was 68%, PFS 60%, and LRFS 70% in the entire cohort. Local failure was seen in 22.7% of patients after radiation. On Cox multivariable analysis incorporating histology, grade, surgical margins, and tumor size at presentation, significant associations were seen between: surgical margin status (R0 vs R1) and LRFS (HR, 4.8; p=0.016); grade and PFS (79% for G1 vs. 29% for G3 at 5 years; HR 3.6; p=0.043); and histology and OS (78% for conventional CS vs 21% for dedifferentiated CS, HR 3.5, p=0.041), PFS (68% vs 20% at 5 years; HR, 5.8; p=0.003), and DMFS (90% vs. 20% at 5 years; HR 19.1; p<0.006).
Conclusion: In this large, single-institution cohort of patients with localized CS, surgical margins status, histologic subtype, and grade were key determinants of outcome. Whereas risk of local recurrence was present across grade and histologic subtype, but was strongly associated with surgical margin status, risk of distant metastasis and overall survival was highly associated with dedifferentiated histology and high-grade pathology. These findings support aggressive pursuit of negative-margin surgical resection and risk-adapted, subtype-specific adjuvant therapy strategies, and highlight the need for prospective studies evaluating the role of systemic and radiation therapies in high-risk CS.