Purpose: The role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS patients and investigated the association between CT/RT with recurrence and survival. Patients and methods: Retrospective review at 25 international sarcoma centers identified patients ≥18 years old treated for ESOS from 1971 to 2016. Patient/tumour characteristics, treatment, local/systemic recurrence, and survival data were collected. Kaplan-Meier survival and Cox proportional-hazards regression and cumulative incidence competing risks analysis were performed. Results: 370 patients with localized ESOS treated definitively with surgery presented with mainly deep tumours (n = 294, 80%). 122 patients underwent surgical resection alone, 96 (26%) also received CT, 70 (19%) RT and 82 (22%) both adjuvants. Five-year survival for patients with localized ESOS was 56% (95% CI 51%-62%). Almost half of patients (n = 173, 47%) developed recurrence: local 9% (35/370), distant 28% (102/370) or both 10% (36/370). Considering death as a competing event, there was no significant difference in cumulative incidence of local or systemic recurrence between patients who received CT, RT, both or neither (local p = 0.50, systemic p = 0.69). Multiple regression Cox analysis showed a significant association between RT and decreased local recurrence (HR 0.46 [95% CI 0.26-0.80], p = 0.01). Conclusion: Although the use of RT significantly decreased local recurrences, CT did not decrease the risk of systemic recurrence, and neither CT, nor RT nor both were associated with improved survival in patients with localized ESOS. Our results do not support the use of CT; however, adjuvant RT demonstrates benefit in patients with locally resectable ESOS.

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Background. Pediatric bone and soft tissue sarcomas are rare; therefore, national registries are essential tools for orthopedic oncology research. Past studies provide excellent data on long-term prognosis and survival trends but fail to examine treatment-specific morbidity. The aim of this study is to use a national registry to describe patient demographics, comorbidities, and adverse events in the first thirty days following surgical management of pediatric bone and soft tissue sarcomas. Methods. A retrospective review of patients in the American College of Surgeons National Surgical Quality Improvement Program—Pediatrics database (NSQIP-P) was performed. The cohort was partitioned by tumor origin (bone versus soft tissue) and tumor location (axial versus appendicular). Results. One-hundred ninety-two patients were identified. Bone sarcomas were more common (71.9%) and predominately appendicular (62.3%), while soft tissue sarcomas were predominately axial (77.8%). The overall complication rate was 8.9%. The most frequent etiologies were wound dehiscence (3.6%) and infectious complications such as surgical site infections (2.6%), pneumonia (1.6%), urinary tract infections (1.6%), and C. diff colitis (1.0%). Twenty-four percent of patients experienced bleeding requiring transfusion. The unplanned readmission rate was 12.5% (3.6% related to principle procedure), and the unplanned reoperation rate was 4.7% (4.2% related to principle procedure). The mortality rate was 1.0%. Neoadjuvant chemotherapy was associated with higher rates of wound dehiscence and infectious complications. There were no differences in adverse events with respect to tumor origin or location. Conclusion. Approximately 1 in 11 pediatric patients will experience a complication in the first thirty days following surgery. However, perioperative mortality remains low. This study represents the first comprehensive review of pediatric bone and soft tissue sarcoma surgery in the NSQIP-P database. As the case volume of NSQIP-P continues to grow, NSQIP-P has the potential to become a powerful tool for pediatric orthopedic oncology research.

Background. Primary bone and soft tissue sarcomas are rare tumors requiring wide surgical resection and reconstruction to achieve local control. Postoperative complications can lead to delays in adjuvant therapy, potentially affecting long-term oncologic outcomes. Understanding postoperative complication risks is essential; however, past studies are limited by small sample sizes. Purpose. This study uses a large national registry to characterize the incidence of complications and mortality in the first thirty days following surgical management of primary bone and soft tissue sarcomas of the extremities. Methods. A retrospective review of patients in the National Surgical Quality Improvement Program database was performed. Cases were identified using diagnosis codes for malignant neoplasm of soft tissue or bone and procedure codes for amputation and radical resection. The cohort was subdivided by bone versus soft tissue sarcoma, upper versus lower extremity, and amputation versus limb salvage. Results. One thousand, one hundred eleven patients were identified. The most frequent complications were surgical site infections, sepsis, and venous thromboembolism. The overall incidence of complications was 14.0%. Unplanned readmission and reoperation occurred after 7.0% and 8.0% of cases, respectively. Thirty-day mortality was 0.3%, with one intraoperative death. Patient factors and complication rates varied by tumor location and surgical modality. Lower extremity cases were associated with higher rates of wound complications and infectious etiologies such as surgical site infections, urinary tract infections, and systemic sepsis. In contrast, patients undergoing amputation were more likely to experience major medical complications including acute renal failure, cardiac arrest, and myocardial infarction. Conclusion. Approximately 1 in 7 patients will experience a complication in the first thirty days following surgery for primary bone and soft tissue sarcomas of the extremities. The unique risk profiles of lower extremity and amputation cases should be considered during perioperative planning and surveillance.

To develop and validate an Osseous Tumor Reporting and Data System (OT-RADS) with the hypothesis that the proposed guideline is reliable and assists in separating benign from malignant osseous tumors with a good area under the curve, and that could assist further patient management. Methods:In this multireader cross-sectional validation study, an agreement was reached for OT-RADS categories based on previously described magnetic resonance imaging features and consensus of expert musculoskeletal radiologists. World Health Organization classification was used, and a wide spectrum of benign and malignant osseous tumors was evaluated. Magnetic resonance imaging categories were as follows: OT-RADS 0-incomplete imaging; OT-RADS I-negative; OT-RADS IIdefinitely benign; OT-RADS III-probably benign; OT-RADS IVsuspicious for malignancy or indeterminate; OT-RADS V-highly suggestive of malignancy; and OT-RADS VI-known biopsy-proven malignancy or recurrent malignancy in the tumor bed. Four blinded readers categorized each tumor according to OT-RADS classification. Intraclass correlation (ICC) and Conger κ were used. Diagnostic performance measures including area under the receiver operating curve were reported. Osseous Tumor Reporting and Data System was dichotomized as benign (I-III) and malignant (IV and V) for calculating sensitivity and specificity.Results: Interreader agreement for OT-RADS (ICC = 0.78) and binary distinction of benign versus malignant (κ = 0.67) were good to excellent, while agreement for individual tumor feature characteristics were poor to fair (ICC = 0.25-0.36; κ = 0.16-0.39). The sensitivities, specificities, and area under the receiver operating curve of the readers ranged from 0.93-1.0, 0.71-0.86, and 0.92-0.97, respectively.Conclusions: Osseous Tumor Reporting and Data System lexicon is reliable and helps stratify tumors into benign and malignant categories. It can be practically used by radiologists to guide patient management, improve multidisciplinary communications, and potentially impact outcomes.