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Cutaneous angiosarcomas (AS) are uncommon and morphologically heterogeneous. Recently, a distinctive lymphatic-type AS with prominent lymphocytic infiltrate has been observed. Although conventional AS typically bear poor prognosis, lymphatic-type AS with prominent lymphocytic infiltrate and pseudolymphomatous AS show prolonged survival with rare extracutaneous spread. We describe a unique case of AS in a 55-year-old woman who received surgical resection and radiation therapy for her prior myxoid liposarcoma. She developed a suspected recurrence 15 years later. Microscopically, the lesion showed an infiltration of the reticular dermis by irregular interanastamosing vascular spaces lined by atypical endothelial cells with nuclear "hobnailing" and hyperchromasia. A prominent intratumoral and peritumoral lymphocytic infiltrate obscuring the tumor cells was also present. The tumor cells were diffusely positive for endothelial cell markers, including D2-40. Notably, there was no evidence of MYC gene amplification by FISH. Additional NGS-based molecular analysis demonstrated no significant genetic mutations. The patient is alive with a history of two local recurrences, but no evidence of metastasis. We present this case to raise awareness of MYC-nonamplified secondary lymphatic-type AS with prominent lymphocytic infiltrate (pseudolymphomatous AS) and to discuss its differential diagnosis.

Introduction Large high-grade sarcomas are commonly managed with five weeks of pre-operative radiation with chemotherapy followed by surgical resection. Wound complications occur in about one out of three patients with this regimen. Hypofractionated radiation therapy (HFRT) is a developing pre-operative approach that delivers radiation over a shorter duration of 5–10 treatments. Presentation of case Two patients underwent HFRT with neoadjuvant chemotherapy followed by tumor resection. The first patient had high-grade de-differentiated liposarcoma, and the second patient a high-grade myxofibrosarcoma. Neither patient developed post-operative wound complications despite the massive tumor size. Discussion Less is understood regarding rates and risk factors associated with wound complications using this shortened radiation approach. With attention to surgical detail, and advancing radiation delivery technologies, rates of complications can be minimized. Conclusion We discuss our experience with a neoadjuvant hypofractionated chemoradiation protocol in two patients with large volume sarcomas resected from the chest wall and the thigh who did not develop acute wound complications. Further evaluation of this shortened regimen is warranted., Highlights • Wound complications are common after sarcoma resection with preoperative radiation. • Hypofractionated Radiation Therapy (HFRT) may be a safe alternative to conventional dose radiation. • HFRT can be utilized with massive volume sarcomas safely.

Noonan syndrome is a common autosomal dominant disorder associated with an increased risk of malignancy. We report a 16-year-old female with Noonan syndrome (KRAS gene variant, Q22R) and diffuse-type tenosynovial giant cell tumor, a proliferative disorder that has been rarely reported in this population. These tumors may represent a complication of the dysregulated RAS/MAPK signaling pathway that underlies Noonan syndrome. They lack typical clinical features, causing misdiagnosis and delays in management, which could lead to osseous invasion requiring more complicated surgical procedures. Increased awareness of this association will improve the clinical outcomes of patients with Noonan syndrome who develop diffuse-type tenosynovial giant cell tumors.

The technique of cryosurgery has been used to control local recurrence in a variety of benign and malignant bone tumors. Early studies revealed significant complication rates (25%) that included fracture, infection, and soft tissue injury. Our method of cryosurgery has yielded excellent tumor control with improved complication rates. The objective of this study is to determine the characteristics of postoperative complications after pouring liquid nitrogen into curettaged bone defects, and to review our current indications and surgical technique in bone tumor management. We reviewed charts in over 200 patients who received cryoablation for bone tumors from 1994 to 2015. Imaging studies were evaluated in all patients diagnosed with a complication. All patients receiving cryotherapy had soft tissue management intraoperatively that included warm saline directed to the structures. Liquid nitrogen was poured into the bone defect and in some cases, additional spraying with a cryogun into the defect was performed. The majority of cryotherapy was used in cases of active or aggressive benign tumors. Our low complication rate of 2.34% included 1 post-operative fracture, 3 infection, and 1 paraesthesia. Bone graft or cementation was used in the majority of patients, all of which fully incorporated. Cryoablation is an excellent from of adjuvant therapy for active and aggressive benign tumors and may be used in malignant tumors as well. Soft tissue protection is critical to avoid skin necrosis and wound breakdown. We recommend the use of cryotherapy in active and aggressive bone tumors as an adjuvant treatment prior to bone grafting or cementation., Highlights • Cryoablation as an adjuvant treatment is recommended in active and aggressive benign tumors. • A combination of a slow freeze and quick thaw, repeated for two cycles giving optimal penetration and stability across structures. • Soft tissue management with warm saline is critical in preventing intraoperative injury. • Controlled cryotherapy techniques can limit complication rates and local recurrence rates.

Case A 72-year-old man presented 20 years after a Morel-Lavallee (ML) lesion with pain and drainage. Biopsies of the lesion and lymph nodes were positive for squamous cell carcinoma (SCC). There was no cutaneous involvement or distant metastasis. After chemotherapy and radiation, he underwent resection of the lesion and lymph nodes with flap closure. Two months postoperatively, he unfortunately developed malignant pleural effusions, hypercalcemia, and kidney injury and was eventually transferred to hospice care and died. Conclusion This is the first report of SCC arising from a ML lesion. Chronic ML lesions should be treated aggressively and monitored for transformation into malignancy, even without cutaneous involvement.

In the original publication of this article [1], there was a mistake in Figure 2. Figure 2a and Fig 2c should be swapped. The revised Figure 2 is shown below.

Background Curettage is widely used in orthopedic oncology; the defect created frequently requires filling for mechanical and functional stability for the bones and adjacent joint. Allograft, bone graft substitute, and polymethyl methacrylate (PMMA) are the most common substances used each with their benefits and drawbacks. The aim of the study is to show that good functional result can be achieved with curettage and bone filler, regardless of type. Methods A series of 267 cases were reviewed between 1994 and 2015 who received curettage treatment and placement of a bone filler. Endpoints included fracture, infection, cellulitis, pulmonary embolism, and paresthesia. Complication rates at our single institution were compared against literature values for three study cohorts: allograft, bone graft substitute, and PMMA bone fillers. Friedman test, Wilcoxon test, and Z-score for two populations were used to compare our subset against literature values and between different bone filling types. Results Our cases included 18 autografts, 74 allografts, 121 bone graft substitute, and 54 PMMA of which the bulk of complications occurred. Our overall complication rate was 3.37%. Allograft has a complication rate of 1.35%, bone graft substitute of 4.13%, and PMMA of 5.56%. Other techniques did not yield any complications. Combination filling techniques PMMA + allograft and PMMA + bone graft substitute had sample sizes too small for statistical comparison. Statistical comparison yielded no significant difference between complications in any of the filling groups (P = 0.411). Conclusions Some has even argued that bone defects following curettage do not require bone filling for good outcome. However, many structural or biologic benefits that aid in earlier return to functionality can be conferred by filling large bone defects. There was no significant difference in postoperative complication rates between allograft, bone graft substitute, and PMMA when compared at our institution and with literature values. Nevertheless, one complication with a large defect filled with allograft, requiring a subsequent reconstruction using vascularized fibular graft. Taking everything into account, we see bone graft substitute as a suitable alternative to other bone filling modalities.

Highlights • Distal fibula resection is a procedure that has been described as early as 1938 for the treatment of neoplastic lesions. • Medial instability of the elbow can be traced back to the literature as far as 1946. • The described technique can preserve long-term tibiotalar congruity and stability, allowing these patients to return to near normal function., Distal fibula resection is a procedure that has been described as early as 1938 for the treatment of neoplastic lesions. There have been several techniques described for the reconstruction of the remaining tibiotalar joint to prevent deformity and maintain as much function as possible. While these reconstruction techniques provide an option for limb salvage with the removal of disease, patients are faced with chronic pain, loss of function, valgus instability, need for long term orthosis, early arthritis, or significant morbidity related to proximal dissection and disruption of native knee anatomy. We present a case series that is compliant with PROCESS1 criteria to demonstrate the effective treatment with distal fibular excision and a reconstruction technique inspired by the original Tommy John procedure of the elbow. This procedure has allowed these two patients long-term stability of the ankle, maintenance of full function, and high levels of function. The first case is a 23-year-old female with high-grade osteosarcoma and the second patient is a 19-year-old female with Ewing sarcoma. Details of the procedure as well as clinical and radiographic follow up of these two patients will be described.

OBJECTIVE The aim of this study was to identify and discuss operative nuances utilizing image guidance in the surgical management of aggressive sacral tumors. METHODS The authors report on their single-institution, multi-surgeon, retrospective case series involving patients with pathology-proven aggressive sacral tumors treated between 2009 and 2016. They also reviewed the literature to identify articles related to aggressive sacral tumors, their diagnosis, and their surgical treatment and discuss the results together with their own experience. Information, including background, imaging, treatment, and surgical pearls, is organized by tumor type. RESULTS Review of the institutional records identified 6 patients with sacral tumors who underwent surgery between 2009 and 2016. All 6 patients were treated with image-guided surgery using cone-beam CT technology (O-arm). The surgical technique used is described in detail, and 2 illustrative cases are presented. From the literature, the authors compiled information about chordomas, chondrosarcomas, giant cell tumors, and osteosarcomas and organized it by tumor type, providing a detailed discussion of background, imaging, and treatment as well as surgical pearls for each tumor type. CONCLUSIONS Aggressive sacral tumors can be an extremely difficult challenge for both the patient and the treating physician. The selected surgical intervention varies depending on the type of tumor, size, and location. Surgery can have profound risks including neural compression, lumbopelvic instability, and suboptimal oncological resection. Focusing on the operative nuances for each type can help prevent many of these complications. Anecdotal evidence is provided that utilization of image-guided surgery to aid in tumor resection at our institution has helped reduce blood loss and the local recurrence rate while preserving function in both malignant and aggressive benign tumors affecting the sacrum.

OBJECTIVE. Our purpose is to present normal and abnormal imaging findings associated with endoprosthetic reconstruction after limb-salvage surgery. CONCLUSION. Endoprosthetic reconstruction varies with the location and size of the tumor, implant designs, and complications. Radiologists need to be aware of associated imaging findings seen in postoperative infection, tumor recurrence, and hardware failure. With a thorough understanding of the normal postoperative radiographic findings after complex reconstructions, subsequent abnormalities are readily identified and timely diagnosis can be obtained.

Giant cell tumor of bone (GCTB) is a rare primary bone tumor that primarily affects young adults, but can be seen in children. The primary modality of treatment is surgical resection; however, this is not always possible given the location and extent of the neoplasm. Recent developments in the understanding of the underlying molecular pathogenesis of disease have pointed to interactions between the stromal component producing receptor activator of nuclear factor-kappaB (RANK) and RANK-ligand (RANKL) causing the formation of osteoclast-like giant cells that drive bone destruction. The development of a monoclonal humanized antibody to RANKL, denosumab, has been shown to reduce skeletal-related events from osteoporosis and from bony metastases from solid tumors. Recent phase II clinical trials with denosumab in skeletally mature adolescents over age 12 years and adults with GCTB, have shown both safety and efficacy, leading to its accelerated US FDA approval on 13 June 2013. In children who are skeletally immature, safety and efficacy has not been established, and there has been only published anecdotal use.

OBJECTIVE The use of intraoperative stereotactic navigation has become more available in spine surgery. The authors undertook this study to assess the utility of intraoperative CT navigation in the localization of spinal lesions and as an intraoperative tool to guide resection in patients with spinal lesions. METHODS This was a retrospective multicenter study including 50 patients from 2 different institutions who underwent biopsy and/or resection of spinal column tumors using image-guided navigation. Of the 50 cases reviewed, 4 illustrative cases are presented. In addition, the authors provide a description of surgical technique with image guidance. RESULTS The patient group included 27 male patients and 23 female patients. Their average age was 61 ± 17 years (range 14–87 years). The average operative time (incision to closure) was 311 ± 188 minutes (range 62–865 minutes). The average intraoperative blood loss was 882 ± 1194 ml (range 5–7000 ml). The average length of hospitalization was 10 ± 8.9 days (range 1–36 days). The postoperative complications included 2 deaths (4.0%) and 4 radiculopathies (8%) secondary to tumor burden. CONCLUSIONS O-arm 3D imaging with stereotactic navigation may be used to localize lesions intraoperatively with real-time dynamic feedback of tumor resection. Stereotactic guidance may augment resection or biopsy of primary and metastatic spinal tumors. It offers reduced radiation exposure to operating room personnel and the ability to use minimally invasive approaches that limit tissue injury. In addition, acquisition of intraoperative CT scans with real-time tracking allows for precise targeting of spinal lesions with minimal dissection.

10528 Background: GCTB are aggressive osteolytic tumors, which are characterized by local bone destruction and soft tissue invasion. There have been limited non-surgical treatments, including radiation therapy. However, an unacceptable rate of post radiation sarcomas has been well established. Such tumors have osteoclast like giant cells/stromal cells that express surface RANK ligand. Denosumab is an FDA approved drug used in the treatment of unresectable GCTB or GCTB with severe morbidity related surgery. It is a monoclonal antibody that targets the RANK ligand characteristic to GCTB. We report our 8-year experience in the treatment of complex GCTB using denosumab. Methods: Review of 43 skeletally mature patients (N = 43) who were treated with subcutaneous (SC) denosumab for the treatment of GCTB, in whom surgery was not feasible without severe morbidity. Denosomab was dosed at 120 mg on days 1, 8, 15, 29, and every 4 weeks thereafter. CT, PET/CT, or MRI was used to determine radiographic disease burden,...

The rare benign extra-abdominal desmoid tumor is characterized by aggressive invasion of normal tissue. Treatment is complicated by its recurrence, invasiveness, and persistence. The etiology is unknown and the pathophysiology is obscure. Because of exuberant fibroblastic proliferation with collagenous tissue being the primary tissue component, this desmoid tumor has been compared with keloids arising from excessive scar formation in healing wounds. Numerous cytokines are associated with signaling for growth and maintenance of mesenchymal cells. Altered expression of these proteins is associated with many pathologic conditions. It has been proposed that the enhanced expression of platelet-derived growth factor and its receptor characterize desmoid tumors. We tested the hypothesis that the exuberant fibrosis of desmoid tumors may have resulted from the initiation of the cascade of molecular events producing increased expression of cytokines. We used immunohistochemical analysis of cytokines in desmoid tumors compared with keloids and skin to localize the expression of cytokines. The results showed localized increased expression of the cytokines epidermal growth factor, transforming growth factor-beta, tumor necrosis factor-alpha, vascular endothelial growth factor, interleukin-1beta, and interleukin-6 in the endothelial cells of blood vessels in the tumors. Production of tumor necrosis factor-alpha and interleukin-1beta in tumor tissue was increased, but we did not find increased expression of platelet-derived growth factor. We concluded that the increased expression of cytokines associated with angiogenesis usually found in wound healing and invasive tumors may contribute to the pathophysiology of the desmoid tumor.