Characteristics and oncologic outcomes of patients with Ewing sarcoma of the scapula.

BACKGROUND AND OBJECTIVES: Ewing sarcoma is the second most common bone sarcoma of childhood. Ewing sarcomas of the scapula are rare, with little known about their characteristics and outcomes. In this study, we describe the demographic characteristics, tumor characteristics, and oncologic outcomes of patients with Ewing sarcoma of the scapula. METHODS: This is a retrospective case series of thirty-four patients treated at three urban hospitals between 1993 and 2014 for Ewing sarcomas affecting the scapula. Their demographic data, tumor characteristics, and oncologic outcomes are reported and contrasted with data on Ewing sarcoma described in the literature. RESULTS: Patients in our case series were 59% male. The average age at diagnosis was 16 years. 44% of patients had metastatic disease at presentation. 26% of patients had a tumor size >8 cm in largest dimension at diagnosis. 9 patients in our series had the t (11; 22) translocation present. Patients had a survival rate of 68% at five years. No patients had local recurrence of disease. Compared with findings reported in the literature concerning Ewing sarcoma affecting other locations, patients with Ewing sarcoma of the scapula were slightly older at time of diagnosis, had a lower percentage of tumors with size > 8 cm in largest dimension at presentation, and more commonly had metastatic disease at presentation. Patients in our cohort had a 5-year survival rate of 68%, which is higher than the rate of approximately 55% as reported in the general literature. CONCLUSIONS: In this study, we describe a retrospective case series of thirty-four patients with Ewing sarcomas of the scapula. This is the largest case series to date of Ewing sarcoma affecting this location to our knowledge. These results will contribute to the understanding of the clinical profile and oncologic behavior of Ewing sarcomas affecting the scapula.

Tumors of the scapula: A retrospective analysis identifying predictors of malignancy.

BACKGROUND: Osseous tumors of the shoulder girdle occur most frequently in the proximal humerus, scapula and clavicle. Prior studies have determined that tumors occurring in the scapula have a higher risk of malignancy, but few have looked exclusively at tumors of this site to describe incidence, clinical features and potential predictors of malignancy. As imaging technologies for diagnosis have improved, it is important to re-evaluate if patterns of incidence have changed and if new imaging findings could potentially be predictors of malignancy. METHODS: This retrospective analysis identified 173 patients from a pediatric and an adult hospital between 1990 and 2015. All patients with tumors of the scapula either of bony origin or with bony invasion were included. Diagnosis, tumor location, and patient demographics were collected. Bivariate and logistic regression analyses were performed to identify potential predictors of malignancy. RESULTS: Chondrosarcoma and osteochondroma were the most common malignant and benign tumors, respectively. Benign tumors were more prevalent in the pediatric population. Tumors occurred most frequently in the body of the scapula. Risk for malignancy increased with age (OR 1.09, 95% CI 1.05-1.12), tumors invading multiple sites (OR 10.85, 95% CI 2.37-49.72) and tumors that were lucent (OR 7.02, 95% CI 1.14-43.21) or had a mixed radiographic appearance (OR 11.47, 95% CI 1.36-99.65). CONCLUSIONS: Including neoplasia in the differential diagnosis for shoulder complaints is important because the scapula is a site of increased risk of malignancy. Patterns of incidence and prevalence have not changed in the last years with the use of more advance techniques of imaging studies. Older age, lucent or mixed imaging, and tumors that span multiple sites of the scapula were found to be predictors of malignancy. Additional cross-sectional anatomy studies may be warranted for further evaluation in patients presenting with these characteristics given the higher concern for malignant disease. LEVEL OF EVIDENCE: Level IV, Prognostic Study.

Aneurysmal bone cyst of the clavicle: a series of 13 cases.

BACKGROUND: Aneurysmal bone cyst (ABC) is a benign but locally aggressive bone tumor occurring most commonly in the first 2 decades of life. The clavicle is a rare location for tumors, and ABCs of the clavicle have been sparsely described in the literature. We present the largest known series of ABCs of the clavicle to describe this rare condition. METHODS: Patients were identified retrospectively from an orthopedic oncology database to obtain demographic, diagnostic, and treatment information. A literature search was performed to identify all English language reports of ABC of the clavicle. RESULTS: We identified 13 patients with ABC of the clavicle, 77% of which were in the acromial end. Most patients (77%) presented with pain or swelling or both. The initial treatment in 11 patients was by curettage, with or without allograft bone packing, and 1 underwent resection/reconstruction of the lateral clavicle. Seven patients (58%) had 1 or more recurrences at an average of 6 months, for which 2 were treated with partial resection of the clavicle. CONCLUSIONS: ABC of the clavicle is a rare condition that we found to occur most frequently in the acromial end of the bone. Most patients were initially treated with curettage and showed a higher rate of recurrence than in other locations. The unique anatomy of the clavicle makes resection a feasible and very functional option, especially if the coracoclavicular ligaments can be preserved. However, the potential resulting deformity may cause patients to opt for a less aggressive and, consequently, less effective treatment method.

Synovial sarcoma of the shoulder: A series of 14 cases.

BACKGROUND AND OBJECTIVES: Synovial sarcoma is a rare soft tissue sarcoma with poor long-term prognosis due to late recurrence and metastasis. Synovial sarcoma arises in less than 6% from the shoulder. As a result, there is limited information in the literature about synovial sarcoma of the shoulder (SSS). METHODS: We included all patients treated for SSS at our institution between 1985 and 2013. Medical charts were retrospectively reviewed to collect demographics, information about the clinical course, and outcome. This subgroup was compared to our institution's entire synovial sarcoma patient cohort and the data in the published literature. RESULTS: SSS Patients presented most commonly with pain and a growing mass; the majority of tumors were grade 2 and measured greater than 5 cm. 43% (7) of SSS patients developed metastatic disease and 36% (5) had died at a median follow-up of 64 months (36-127); SSS 5-year survival (83.3%) was higher in our series than in the general literature (57-75%). CONCLUSIONS: We found better prognosis in patients with synovial sarcoma of the shoulder than expected based on the current literature. The clinical behavior of synovial sarcoma in the shoulder is closer to that of synovial sarcoma in the extremities than the trunk. LEVEL OF EVIDENCE: Level IV, Case Series.

Primary arthroplasty in healed osteoarticular allograft in patients with history of primary femoral bone tumors.

BACKGROUND: Roughly 25-35% of patients who are treated with osteoarticular allograft for primary bone sarcomas or aggressive benign bone tumors require surgery in the long-term due to degenerative changes of the articular surface of the allograft. There are three established methods of reconstruction for this complication; a total hip arthroplasty (THA) or total knee arthroplasty (TKA) in the retained osteoarticular allograft, a proximal or distal endoprosthesis after removal of the allograft, and an allograft-prosthesis composite (APC). The aims of this study are 1) to determine the rate of complication and failure of THA/TKA in healed femoral allograft; 2) to compare the methods of revision for allograft degeneration; and 3) to compare the use of arthroplasty in healed allograft to that of arthroplasty in native bone. METHODS: We included all patients with primary bone sarcomas and locally aggressive primary benign bone tumors treated between 1984 and 2014 with an osteoarticular allograft followed by any subsequent arthroplasty technique as described above. Complications and reasons for failure are described following the classification of Henderson et al. Failure was defined as any complication leading to removal of the initial treatment construct. Failure rates of these groups were compared to primary arthroplasty in a live host bone (Control Group). RESULTS: Complications happened in 25 (61.0%) of the patients with a THA/TKA in the retained allograft, of these, 24 (58.5%) experienced failure, the most common being structural failure/type III (14, 58.3%). Thirteen patients (81.3%) with an endoprosthesis after removal of the allograft experienced complications, all of whom failed. The most common failure modes were aseptic loosening/type II (4, 30.8%) and infection/type IV (5, 38.5%). Complications in patients with an APC were experienced by 12 (85.7%) patients, 11 (78.6%) of whom failed. The most common failure mode was infection/type IV (4, 36.4%). Significantly (p < 0.001) fewer failures were observed in the control group compared to patients with an arthroplasty in a healed allograft. CONCLUSIONS: We found no significant difference in the outcome of treating patients with allograft and subsequent degenerative bone disease with a THA/TKA in a retained allograft, an endoprosthesis after removal of the allograft, or a primary APC, although infection is a significantly greater cause of failure in the latter two. Primary arthroplasty in healed allografts is a less extensive surgery than removing the allograft and shows comparable complication and failure rates. LEVEL OF EVIDENCE: Level III, Therapeutic Study.

Predictors of venous thromboembolism in patients with primary sarcoma of bone.

Venous thromboembolism (VTE) is known to be independently associated with both orthopaedic surgery and malignancy. Patients undergoing surgery for musculoskeletal malignancies are at an increased risk for thromboembolic events. Although VTE can lead to serious morbidity and mortality, the potential complications of prophylactic anticoagulation call for a greater understanding of a patient's risk factors, as well as more rigorous guidelines for prophylactic anticoagulation regimens. The aims of this study were to 1) Analyze the rate of VTE in patients surgically treated for primary bone sarcoma; 2) Identify risk factors for VTE in patients with primary bone sarcoma; 3) Discuss the complications associated with prophylactic anticoagulation in patients with primary bone sarcoma. METHODS: This retrospective study identified all patients 18 years and older treated surgically at our institution for a primary bone sarcoma between 1990 and 2015. All patients with at least 90 days of post-operative follow-up from the index surgery were analyzed for occurrence of VTE. Those with an event were compared to those without to identify predictors of VTE. RESULTS: 21 patients (5.5%) had a clinically symptomatic, radiographically confirmed VTE within 90 days of index surgery (12 DVT, 9 PE). Higher preoperative white blood cell count (OR 1.15, 95% CI 1.01-1.29) and post-operative wound complications (OR 5.01, 95% CI 1.93-13.55) were found to be independent risk factors for VTE. No differences in terms of efficacy were found among medications. The risk of wound complications increased significantly in patients who received chemical prophylaxis (OR 2.21, 95% CI 1.00-4.87). CONCLUSIONS: Our patient population had a relatively low rate of VTE as compared to the literature. Preoperative white blood cell count (WBC) and post-operative wound complications were both found to be independently associated predictors for VTE in patients with primary bone sarcoma. An elevated WBC may reflect hemoconcentration which per se is prothrombotic in nature. Aggressive DVT prophylaxis may, counter intuitively, increase the risk of thromboembolic events due to prolonged immobilization and additional surgeries that usually occur when addressing postoperative wound complications such as postoperative hematoma and wound dehiscence that may relate to overanticoagulation. Prospective randomized trials comparing different medications in combination with compressive devices are needed to assess efficacy with the lowest complication profile. Simpler protocols may increase patient compliance to prophylactic treatment.

Cemented Total Hip Arthroplasty With Retrograde Ischioacetabular Steinmann Pin Reconstruction for Periacetabular Metastatic Carcinoma.

BACKGROUND: Surgical management of advanced periacetabular lesions is challenging because of extensive bone loss, particularly for Modified American Academy of Orthopaedic Surgeons Classification defects type IV (pelvic discontinuity with posterior column involvement). Multiple methods for rebuilding the acetabulum have been described; all involve passing Steinmann pins in a retrograde or an antegrade fashion from the anterior iliac wing or iliac crest around the acetabulum in an attempt to recreate the normal bony anatomy. However, these techniques fail to engage the ischium in its entirety. The ischial contribution to the posterior column is a critical element in a stable acetabular construct. METHODS: After curettage of the acetabular lesion, Steinmann pins are passed through the ischial tuberosity and posterior column into the sciatic buttress in a retrograde manner. The number of pins depends on the size of the defect and involvement of the posterior column. PATIENTS AND METHODS: Medical records of 11 patients with a Modified American Academy of Orthopaedic Surgeons Classification defect type IV treated with retrograde ischioacetabular Steinmann pin reconstruction at our institution between 2007 and 2012 were reviewed. European Quality of Life-5 dimensions and Lower Extremity Functional Scale questionnaires were used to assess patient functional outcomes. RESULTS: The 6 patients (4 females and 2 males; age range, 56-81 years) surviving 12 months postoperatively reported improved mobility and good quality-of-life scores. CONCLUSION: We described a new method for posterior acetabular column reconstruction that uses the ischial tuberosity and body as additional points of stabilization during the reconstruction of the posterior column.