Temple, H. Thomas

ABSTRACT Chordomas are rare notochordal malignancies typically occurring in the axial skeleton. Less than 100 extra‐axial chordomas have been reported. We describe a unique chordoma of the anteromedial thigh. The clinical presentation of extra‐axial chordomas is highly variable; thus, recognizing this entity in the differential diagnoses of extra‐axial bone/soft tissue tumors is important.

AbstractBackground and ObjectivesManagement of myxofibrosarcoma (MFS) is widely debated, and there is no consensus regarding the effect of adjuvant therapies on local recurrence (LR), distant metastasis (DM), or overall survival (OS). This study aims to determine the effect of adjuvant therapies on the aforementioned outcomes in patients with MFS.MethodsWe conducted a systematic review via Medline, PubMed, CINAHL, Cochrane, Embase, and Google Scholar. Therapeutic studies that reported LR, DM, and OS following adjuvant therapies in patients with MFS were included. Statistical analysis and creation of hazard ratios were performed using Revman 5.3 (Cochrane Collaboration).ResultsWe identified 301 studies, 10 of which were included in the final analysis. Neither chemotherapy nor radiation affected the rate of LR (p = 0.73 and 0.17, respectively) or OS (p = 0.97 and 0.27, respectively). Chemotherapy was significantly associated with lower rates of DM (p = 0.03); however, there was no association between radiation and DM (p = 0.67).ConclusionThere is a lack of consensus regarding the effect of neoadjuvant and adjuvant therapies on LR, DM, and OS in patients with MFS. Our systematic review found that radiation and chemotherapy were not significantly associated with OS or LR in patients with MFS. However, we identified a significant association between chemotherapy and lower rates of DM.

Previous classification systems of failure of limb salvage focused primarily on endoprosthetic failures and lacked sufficient depth for the effective study of the causes of failure. In order to address these inadequacies, the International Society of Limb Salvage (ISOLS) formed a committee to recommend revisions of the previous systems. The purpose of this study was to report on their recommendations. The modifications were prepared using an earlier, evidence-based model with subclassification based on the existing medical literature. Subclassification for all five primary types of failure of limb salvage following endoprosthetic reconstruction were formulated and a complementary system was derived for the failure of biological reconstruction. An additional classification of failure in paediatric patients was also described. Limb salvage surgery presents a complex array of potential mechanisms of failure, and a complete and precise classification of types of failure is required. Earlier classification systems lacked specificity, and the evidence-based system outlined here is designed to correct these weaknesses and to provide a means of reporting failures of limb salvage in order to allow the interpretation of outcome following reconstructive surgery. Cite this article: Bone Joint J 2014;96-B:1436–40.