Background: Sacral chordoma is a rare, low to intermediate-grade tumor that poses substantial challenges in terms of timely diagnosis and adequate treatment. Few studies have examined the oncologic and functional outcomes of patients treated for sacral chordoma.

➤ Biopsy, staging, preoperative imaging and planning, as well as surgical treatment of soft-tissue sarcomas, are best carried out in specialized sarcoma centers, with the support of a multidisciplinary tumor board. ➤ Tumor bed excision is recommended after most unplanned excisions, with a goal of obtaining complete tumor removal with an appropriately wide margin of resection. ➤ The surgical resection area tends to be more extensive during tumor bed excision than during primary resection because of the need to resect potential areas of contamination, resulting in the need for more reconstructive procedures (flaps and skin grafts) and wider radiation fields. ➤ Unplanned excisions are associated with an increased rate of local recurrence related to residual disease and positive margins after tumor bed excision, a deep location, and certain histologic subtypes, such as malignant peripheral nerve sheath tumor, myxofibrosarcoma, and dermatofibrosarcoma protuberans. ➤ While adjuvant radiation therapy has not been found to mitigate the risk of local recurrence in unplanned excisions, it is generally utilized in the treatment of unplanned excisions as it is in the treatment of primary soft-tissue sarcomas. ➤ Given the surgical and oncologic sequelae of unplanned excisions, prevention through the diffusion of concepts by means of provider education on how to approach soft-tissue masses, which can be potential soft-tissue sarcomas, is the best strategy.

Background: Peripheral nerve sheath tumors (PNSTs) are soft tissue neoplasms found in intimate association with a peripheral nerve. They are rarely seen in the foot and ankle where they have an innocuous appearance, but these tumors have the potential to become malignant. This study reports a large series of foot and ankle PNSTs surgically treated at a single institution. Materials and Methods: Retrospectively, all cases of PNSTs confirmed by biopsy and surgically treated from 1992 to 2008 were included in the study. Preoperative, perioperative and postoperative variables were collected. Foot and ankle tumors were compared with the overall group of PNSTs. Fisher's test and Student's t-test were implemented to address the significance of the findings (p< 0. 05). Results: From all PNSTs identified (n= 137), there were 14 (10.2%) foot and ankle. A painful mass was the most common presenting symptom. Schwannoma represented the most common histologic type. Two cases of malignant foot and ankle PNSTs were identified. Foot and ankle PNSTs were significantly smaller in size and showed fewer postoperative neurologic deficits than the overall group of PNSTs. No differences were found in terms of histologic type distribution, age at diagnosis, duration of symptoms and postoperative tumor recurrence, but there was a preponderance of females in this series of foot and ankle tumors. Conclusion: PNSTs of the foot and ankle usually present as innocuous, slow-growing masses leading to misdiagnosis and suboptimal surgical treatment of a potentially malignant lesion. A comprehensive diagnostic evaluation, preoperative planning and meticulous surgical excision are required to prevent tumor recurrence and minimize the incidence of postoperative sequelae, thus improving postoperative functional outcomes.

Patients undergoing proximal femoral replacement for tumor resection often have compromised hip abductor muscles resulting in a Trendelenberg limp and hip instability. Commercially available proximal femoral prostheses offer several designs with varying sites of attachment for the abductor muscles, however, no analyses of these configurations have been performed to determine which design provides the longest moment arm for the hip abductor muscles during normal function.

Giant cell tumor of tendon sheath and pigmented villonodular synovitis are synovial-based diseases that are generally treated by surgery. For aggressive and recurrent tumors, treatment alternatives are needed. This review explores a targeted therapeutic strategy. Imatinib, a tyrosine kinase inhibitor, blocks expression of colony stimulating factor 1 (CSF1) by a small subpopulation of tumor cells that recruit CSF1-bearing nonneoplastic cells. Limited clinical data support the efficacy and side effect profile of imatinib in stabilizing disease in most patients. Imatinib along with other such inhibitors and monoclonal antibodies to CSF1R are putative drugs that may play an important role in the treatment of these tumors.