Allograft-prosthetic composite reverse total shoulder arthroplasty for reconstruction of proximal humerus tumor resections.

BACKGROUND: Proximal humerus reconstructions after resection of tumors are challenging. Early success of the reverse shoulder arthroplasty for reconstructions has recently been reported. The reverse allograft-prosthetic composite offers the advantage of improved glenohumeral stability compared with hemiarthroplasty for proximal humeral reconstructions as it uses the deltoid for stability. METHODS: This article describes the technique for treating proximal humeral tumors, including preoperative planning, biopsy principles, resection pearls, soft tissue tensioning, and specifics about reconstruction using the reverse allograft-prosthetic composite. Two cases are presented along with the functional outcomes with use of this technique. Biomechanical considerations during reconstruction are reviewed, including techniques to improve the deltoid compression force. RESULTS: Reported instability rates are less with reverse shoulder arthroplasty reconstruction as opposed to hemiarthroplasty or total shoulder arthroplasty reconstructions of tumor resections. Reported functional outcomes are promising for the reverse allograft-prosthetic composite reconstructions, although complications are reported. CONCLUSION: Reverse allograft-prosthetic composites are a promising option for proximal humeral reconstructions, although nonunion of the allograft-host bone junction continues to be a challenge for this technique.

Giant Cell Tumors of the Foot and Ankle Bones: High Recurrence Rates After Surgical Treatment.

Giant cell tumor (GCT) of the bone is a benign primary bone tumor most often treated with intralesional surgery. Most cases occur around the knee; however, rarely, GCT of bone can occur in the foot and ankle. Limited data exist about the outcomes after treatment of GCT in this location. We retrospectively reviewed an orthopedic oncology database from 1970 to 2010 for cases of GCT of the bone, specifically within the foot and ankle bones. After exclusionary criteria were applied, a total of 19 disease sites in 18 patients were included for analysis. Of the 19 disease sites, 10 recurred. Patients, on average, required 1.7 operations per disease site. Of the 18 patients, 10 required ≥2 operations, 3 required ≥3 operations, and 1 required 4 operations. A total of 4 amputations were performed, including 2 below the knee amputations. Of the 10 patients with recurrence, 2 also had evidence of metastatic disease. The recurrence rates of GCT in the foot and ankle bones appear to be greatest after intralesional curettage without the use of cement. Although the recurrence rates are high, intralesional operations with multiple adjuvant therapy can eventually result in cure.

Multidisciplinary management of soft tissue sarcoma.

Soft tissue sarcoma is a rare malignancy, with approximately 11,000 cases per year encountered in the United States. It is primarily encountered in adults but can affect patients of any age. There are many histologic subtypes and the malignancy can be low or high grade. Appropriate staging work up includes a physical exam, advanced imaging, and a carefully planned biopsy. This information is then used to guide the discussion of definitive treatment of the tumor which typically involves surgical resection with a negative margin in addition to neoadjuvant or adjuvant external beam radiation. Advances in imaging and radiation therapy have made limb salvage surgery the standard of care, with local control rates greater than 90% in most modern series. Currently, the role of chemotherapy is not well defined and this treatment is typically reserved for patients with metastatic or recurrent disease and for certain histologic subtypes. The goal of this paper is to review the current state of the art in multidisciplinary management of soft tissue sarcoma.

Effect of Insurance Status on Mortality in Adults With Sarcoma of the Extremities and Pelvis: A SEER-Medicare Study.

INTRODUCTION: Previous studies have highlighted the association between insurance status and poor outcomes after surgical treatment of sarcomas in the United States.1-3 It is unclear how much of this disparity is mediated by confounding factors such as medical comorbidities and socioeconomic status and how much can be explained by barriers to care caused by insurance status. METHODS: Surveillance, Epidemiology, and End Results-Medicare linkage data were procured for 7,056 patients undergoing treatment for bone and soft-tissue sarcomas in the extremities diagnosed between 2006 and 2013. A Cox proportional hazards model was used to assess the relative contributions of insurance status, medical comorbidities, tumor factors, treatment characteristics, and other demographic factors (race, household income, education level, and urban/rural status) to overall survival. RESULTS: Patients with Medicaid insurance had a 28% higher mortality rate over the period studied, compared with patients with private insurance (hazard ratio, 1.28; 95% confidence interval, 1.03 to 1.60, P = 0.026), even when accounting for all other confounding variables. The 28% higher mortality rate associated with having Medicaid insurance was equivalent to being approximately 10 years older at the time of diagnosis or having a Charlson comorbidity index of 4 rather than zero (hazard ratio, 1.27). DISCUSSION: Insurance status is an independent predictor of mortality from sarcoma, with 28% higher mortality in those with pre-expansion Medicaid.4,5 This association between insurance status and higher mortality held true even when accounting for numerous other confounding factors. Additional study is necessary into the mechanism for this healthcare disparity for the uninsured and underinsured, as well as strategies to resolve this inequality.

The significance of a marginal excision after preoperative radiation therapy for soft tissue sarcoma of the extremity.

BACKGROUND: Marginal excision of soft tissue sarcoma (STS), defined as resection through the tumor pseudocapsule or surrounding reactive tissue, increases the likelihood of local recurrence and necessitates re-excision or postoperative radiation. However, its impact after preoperative radiation therapy (RT) remains unclear. This study therefore investigated the significance of marginal margins in patients treated with preoperative RT for extremity STS, reporting long-term local control and limb preservation endpoints. METHODS: The records of 317 adults at the University of Florida with nonmetastatic extremity STS treated from 1980 to 2008 with preoperative RT as part of a limb conservation strategy were reviewed. The median follow-up was 4.7 years (8.3 years for living patients). The median tumor size was 10 cm (range, 2-36 cm), and 86% were high grade. The median RT dose was 50.4 Gy (range, 12.5-57.6 Gy). Margins were classified as wide/radical (n = 105), marginal (n = 179), contaminated (n = 15), positive (n = 17), or unknown (n = 1). Endpoints were local control (LC), amputation-free survival (AFS), cause-specific survival (CSS), and overall survival (OS). RESULTS: Five-year CSS and OS rates were 62% and 59%, respectively. Five-year LC and AFS was 93% and 89%, respectively. AFS by margin status was 64%, 83%, 97%, and 92% for positive, contaminated, marginal, and wide/radical margins, respectively (P<.005). Marginal excision following preoperative RT resulted in equivalent LC and AFS compared with wide/radical margins. CONCLUSIONS: Marginal resection after preoperative RT does not compromise LC or AFS in extremity STS. This finding may be related to radiosterilization of tumor cells within the reactive zone following preoperative RT.

Modern Therapy for Chest Wall Ewing Sarcoma: An Update of the University of Florida Experience.

PURPOSE: Owing to adjacent critical organs, the aggressive multimodality local therapy necessary for Ewing sarcoma of the chest wall is a challenge. Our previous review of historical outcomes at our institution revealed suboptimal disease control and a high incidence of grade ≥3 toxic effects in patients treated before 2006. The purpose of this study was to evaluate changes during the past decade since the introduction of proton therapy. METHODS AND MATERIALS: Thirty-nine consecutive pediatric patients with a chest wall Ewing sarcoma treated between 2006 and 2020 at the University of Florida were identified. The median maximum tumor diameter was 10 cm (range, 4-28 cm). At diagnosis, 19 patients had local disease and the others had a pleural effusion (11), pleural nodules (5), or pulmonary metastases (4). Patients were treated with chemotherapy regimens according to contemporary North American and European protocols: 7 were treated with preoperative, 18 with postoperative, and 14 with definitive radiation. Preceding primary site treatment, 15 patients required hemithorax radiation and 4 patients underwent whole-lung irradiation using photon techniques. The total median radiation dose to the primary tumor was 52.8 GyRBE [relative biological effectiveness] (range, 44.4-55.8 GyRBE). RESULTS: With a median follow-up of 4 years (range, 0.7-14.7 years), the 5-year local control, progression-free survival, and overall survival rates were 97.2%, 74.4%, and 81.6%, respectively, for the whole cohort. For the 19 patients with nonmetastatic disease, the 5-year local control, progression-free survival, and overall survival rates were 100%, 78.9%, and 78.9%, respectively. No patients developed grade ≥4 toxic effects. Two patients (5%) experienced grade 3 toxic effects related to multimodality treatment; both were patients who required surgery to correct scoliosis. Two patients (5%) developed grade 2 pneumonitis. CONCLUSIONS: Compared with our prior published institutional experience, our data suggest improvements in disease control and multimodality toxic effects since the introduction of proton therapy. This should be confirmed with a larger sample size and longer follow-up.

Effect of Resident and Fellow Involvement on Outcomes of Sarcoma Surgery: A NSQIP Database Cross-Sectional Study.

BACKGROUND: The complexity of sarcoma surgery often justifies surgical assistants of higher levels of academic training: senior residents, fellows, or co-surgeons. The association between the level of training of assistants and outcomes of these procedures has yet to be studied. METHODS: The Current Procedural Terminology (CPT) codes comprising the "core" procedures for musculoskeletal oncology fellowships were gathered. After CPTs primarily capturing nononcologic procedures were excluded, the National Surgical Quality Improvement Program (NSQIP) database was used to find procedures with these CPTs. The severity of complications was assessed using the Severity Weighting of Postoperative Adverse Events in Orthopedic Surgery (SWORD) score. Resident/fellow presence was analyzed both as a binary variable and stratified by level of training. RESULTS: In 159 cases meeting inclusion criteria, higher-level assistants were associated with increased rate of any complication (p=0.006) and greater need for transfusion (p=0.001) but also tended to be used in cases of longer duration (p=0.001) and with higher total work relative value units (wRVUs) (p=0.001). Multivariate analysis showed that while higher-wRVU procedures persisted as an independent predictor of increased complications (OR 1.028 per RVU unit, p=0.002), neither the presence nor level of training of assistants had an independent effect on complication rates. Other independent predictors of 30-day complications were treatment comorbidity (OR 3.433, p=0.010) and lower extremity location of the tumor (OR 4.393, p=0.006). Severity of complications did not differ between any of the groups on either univariate or multivariate analysis. CONCLUSIONS: Trainees of higher levels of academic training tend to be present for longer, higher-complexity musculoskeletal oncology cases, but the overall severity of complications from these do not significantly differ from lower-risk cases without trainees. Orthopedic oncologists may reassure patients that the presence of trainees and co-surgeons is not only safe but it may also help reduce the severity of complications in more complex procedures.

Distal femoral osteoarticular allografts: long-term survival, but frequent complications.

BACKGROUND: Complications are frequent with osteoarticular allografts, and their long-term survivorship in the distal femur is unclear. Thus, the benefits of osteoarticular allografting remain controversial. QUESTIONS/PURPOSES: We therefore determined the frequency of complications in osteoarticular allografts of the distal femur relative to their potential long-term survival. METHODS: We retrospectively reviewed 26 patients who had osteoarticular allograft reconstruction of the distal femur after resection of a malignant or aggressive benign tumor of bone. The minimum followup was 15 months (average, 156 months; range, 15-283 months) for all patients and 98 months (average, 191 months; range, 98-283 months) for the surviving patients. RESULTS: At last followup, 16 of the 26 original allografts were still in place. The overall 5-year and 10-year allograft survival rates were 69% and 63%, respectively. The 5-year and 10-year survival rates of the joint surface were 79% and 65%, respectively. Eleven patients retained their original osteoarticular allograft without a resurfacing procedure, and nine had been converted to allograft-prosthetic composites. Five patients were converted to megaprostheses and one had an amputation for local recurrence. At last followup, 25 of 26 patients retained a functional limb. CONCLUSIONS: Osteoarticular allograft reconstructions of the distal femur can provide long term survival and restore function but the risk of complications and their physical and monetary costs for patients are not trivial. Lacking the benefit of improved soft tissue attachments inherent in other anatomic sites, we believe this option is most appropriate for restoring bone stock in young patients with expectations of long-term survival.

Radiotherapy for soft tissue sarcoma of the proximal lower extremity.

Soft-tissue sarcoma (STS) is a histopathologically diverse group of tumors accounting for approximately 10,000 new malignancies in the US each year. The proximal lower extremity is the most common site for STS, accounting for approximately one-third of all cases. Coordinated multimodality management in the form of surgery and radiation is often critical to local control, limb preservation, and functional outcome. Based on a review of currently available Medline literature and professional experience, this paper provides an overview of the treatment of STS of the lower extremity with a particular focus on the modern role of radiotherapy.

Pediatric Sarcoma Patients with Worse Physical Function but Better Peer Relationships and Depressive Symptoms than U.S. Pediatric Population as Measured by PROMIS®.

Background Pediatric patients with sarcomas are at risk of poor quality of life outcomes. The National Institutes of Health (NIH)-funded Patient-reported Outcomes Measurement Information System (PROMIS®) improves our ability to capture patient-reported outcomes. Do physical function, social, and mental health PROMIS outcomes for pediatric patients with non-metastatic malignant sarcomas differ from the U.S. pediatric population? Methods Six pediatric PROMIS short forms were collected for patient visits to orthopedic oncology at a tertiary referral center from September 1, 2016, to March 31, 2017. Mean T-scores differed from the reference population by a one-sample t-test. Results Of the 30 eligible patients, five had soft-tissue sarcomas and 25 (83%) had bone sarcomas. The mean age of the cohort was 13 years (5-17). The study cohort had a mean physical function T-score of 39.8 (SD 9.8), which was worse than the reference population. In contrast, the mean peer relationship T-score of 54.3 (SD 8.8) and mean depression T-score of 42.0 (SD 9.1) were better than the reference population. Conclusions Pediatric patients with non-metastatic sarcomas had a worse physical function but a better peer relationship and depression scores than the U.S. PROMIS reference population. Ceiling and flooring effects were reported. The level of evidence was III.

Brain metastases from Truncal and extremity bone and soft tissue sarcoma: Single institution study of oncologic outcomes.

Brain metastases are a rare occurrence in patients with sarcoma. The prognosis for patients is poor, and treatment can contribute to considerable morbidity. We sought to examine the experience of our institution in managing these patients over a period of 17 years. We performed a retrospective cohort study of patients managed for sarcoma of the extremity or trunk who developed brain metastases from 2000 to 2017. Clinical data were analyzed and we assessed survival outcomes. 14 patients presenting at a mean age of 46.7 years were included. All patients were treated with radiotherapy for their brain metastases. 3 patients underwent surgical excision of their intracranial metastases. Two patients were treated with radium-223 dichloride. Kaplan-Meier survival analysis and the log rank test were used to calculate the survival probability, and to compare patient subgroups. All patients in this study developed lung or bone metastases at a mean interval of 13.3 months prior to the development of brain metastasis. The median interval from diagnosis of a brain metastasis to death was 3.6 months. The Kaplan-Meier survival probability at 6 months was 28.6%, and 14.3% at 1 year. Surgery was not found to be associated with increased survival. Patients with cerebellar metastasis had increased survival probability as compared to those with cerebral metastasis. Patients with extremity or trunk sarcoma who develop brain metastases frequently develop lung or bone metastases in the year preceding their diagnosis of brain metastasis. Patients with cerebellar metastasis may have better survival than those with cerebral metastasis, and an aggressive treatment approach should be considered. Despite aggressive treatment, the prognosis is grim.

Infarct-associated bone sarcomas.

UNLABELLED: Sarcoma associated with bone infarct is a rare condition sparsely reported in the literature. Sixty percent of cases arise about the knee and most are malignant fibrous histiocytomas. We report 15 patients; 12 of 15 presented with a tumor around the knee. Treatment was limb salvage in seven patients, amputation in six, and biopsy alone in two. For patients without metastatic disease at presentation, the 2-year disease-free survival rate was 63% (seven of 11). Two patients received chemotherapy and both were continuously disease-free at last followup. When we combined our 15 patients with the 52 previously reported in the literature, 38 of the 67 (57%) died of their disease at an average of 19.2 months after diagnosis; 21 patients (31%) were continuously disease-free for 24 months. Of 13 patients who received chemotherapy, eight (62%) were continuously disease-free at 24 months compared with 24% (13 of 54) of those who did not receive chemotherapy. Overall, prognosis for these patients is poor, but survival in patients without metastatic disease at diagnosis approaches that of other bone sarcomas. There is a trend suggesting adjuvant chemotherapy combined with appropriate surgery may improve patient outcomes. LEVEL OF EVIDENCE: Level IV, therapeutic study.

An Evaluation of PROMIS Health Domains in Sarcoma Patients Compared to the United States Population.

BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a patient-directed system that allows comparisons across medical conditions. With this tool, comparisons can now be made between rare conditions, such as sarcomas, and more common ailments, of the United States general population. This allows comparisons between rare conditions, such as sarcomas, to more common ailments, or even the United States (US) general population. OBJECTIVES: Our purpose was to use PROMIS to compare outcomes in patients that had undergone resection of a nonmetastatic sarcoma to the US population. METHODS: One hundred thirty-eight patients were included in the analysis. These patients were divided into early (<2 years) and late follow-up (>2 years). RESULTS: We evaluated results from seven health domains and found significantly lower scores in the physical function and depression domains. These differences were present in both the early and late cohorts when compared to the US population. CONCLUSION: While physical function was found to be worse in the sarcoma cohorts, we observed significantly improved levels of depression in these patients when compared to the US population. This finding was maintained over time and is an important reminder that a patient's goals and desires change following a cancer diagnosis and must be taken into consideration when planning treatment and determining a successful outcome.

Evaluation of Planned versus Unplanned Soft-Tissue Sarcoma Resection Using PROMIS Measures.

BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a tool developed by the National Institutes of Health that allows comparisons across conditions or even the United States (U.S.) general population. OBJECTIVES: Our purpose was to compare PROMIS outcomes between patients who underwent a planned resection to those who underwent an initial unplanned excision of their sarcoma followed by a definitive oncologic resection. We then compared these groups to the U.S. general population. METHODS: Eighty-five patients were included and were divided into those who underwent an initial planned resection (67) and unplanned excision (18). These patients were then further categorized based on the length of follow-up since their last surgery, either early (<12 months) or late (>12 months). RESULTS: We evaluated seven PROMIS domains and found no differences between patients who underwent planned resection versus those who underwent an initial unplanned excision followed by a wide resection of the previous wound bed. When compared to the U.S. population, both cohorts demonstrated significantly improved scores in several emotional health domains. CONCLUSIONS: Patients who undergo an unplanned excision followed by a definitive oncologic procedure have similar PROMIS scores compared to patients who undergo an initial planned resection.

Impact of local management on long-term outcomes in Ewing tumors of the pelvis and sacral bones: the University of Florida experience.

PURPOSE: This retrospective analysis describes our 35-year experience with respect to disease control and functional status. PATIENTS AND METHODS: Thirty-five patients with localized Ewing tumors of the pelvis and sacral bones were treated from 1970 to 2005. Twenty-six patients were treated with definitive radiotherapy (RT), and 9 patients were treated with combined local therapy in the form of surgery + RT. The median RT dose was 55.2 Gy. The patients who received RT alone were more likely to be older men with larger tumors exhibiting soft-tissue extension. Patients in the definitive RT group were more likely to receive etoposide and ifosfamide or undergo bone marrow transplant. Median potential follow-up was 19.4 years. RESULTS: The 15-year actuarial cause-specific survival, freedom from relapse rate, and local control rates were 26% vs. 76% (p = 0.016), 28% vs. 78% (p = 0.015), and 64% vs. 100% (p = 0.087), respectively, for patients treated with definitive RT and combined therapy. Overall, tumors <8 cm had significantly better cause-specific survival, but this was unrelated to local control. The median Toronto Extremity Salvage Score for the definitive RT and combined therapy groups were 99 and 94, respectively (p = 0.19). Seven definitive RT patients (27%) had serious complications. CONCLUSION: Combined modality local therapy should be considered if pelvic Ewing tumors are resectable. However, because of the extent of local disease, most patients have unresectable or partially resectable tumors and therefore require RT in some capacity. For this reason, innovative RT strategies are needed to improve long-term disease outcomes and minimize side effects while maintaining an acceptable functional result.

Establishment and characterization of OS 99-1, a cell line derived from a highly aggressive primary human osteosarcoma.

Osteosarcoma is the most common form of primary bone cancer. In this study, we established a human osteosarcoma cell line (OS 99-1) from a highly aggressive primary tumor. G-banding karyotype analysis demonstrated a large number of clonal abnormalities, as well as extensive intercellular heterogeneity. Through the use of immunologic, molecular, and biochemical analyses, we characterized protein and gene expression profiles confirming the osteogenic nature of the cells. Further evaluation indicated that OS 99-1 cells maintain the capacity to differentiate in an in vitro mineralization assay as well as form tumors in the in vivo chicken embryo model. This cell line provides a useful tool to investigate the molecular mechanisms contributing to osteosarcoma and may have the potential to serve as a culture system for studies involving bone physiology.

Reverse-reamed Intercalary Allograft: A Surgical Technique.

Allograft reconstruction of large segmental intercalary bone defects after tumor resection is a well-accepted surgical technique. Although results generally have been satisfactory, nonunion at the allograft-host bone junction site remains a notable concern. Various reports have described attempts to enhance junctional healing with a variety of complex osteotomies, often complicating an already complex procedure. The use of an innovative reverse reaming technique can decrease the level of intraoperative difficulty mating the allograft-host junction and theoretically may improve junctional healing by enhancing stability and increasing the contact surface area.

Diffuse Pigmented Villonodular Synovitis as a Rare Cause of Graft Failure Following Anterior Cruciate Ligament Reconstruction.

This case report describes a 42-year-old woman who was diagnosed with pigmented villonodular synovitis (PVNS) in the knee. The patient had received a bone-patella tendon-bone autograft reconstruction of her anterior cruciate ligament (ACL) 22 years prior to her diagnosis of PVNS. After a traumatic event that tore her ACL graft, she underwent a second surgery to repair the initial reconstruction. However, her pain and joint instability remained unresolved. When radiolucent lesions in her tibia and femur were identified through a radiographic image, the patient was referred to the authors' orthopedic oncology clinic. Additional imaging, including magnetic resonance imaging, revealed PVNS, and she was scheduled for debridement and a complete synovectomy of the knee. After surgery, the patient's pain decreased dramatically. She continues to maintain an active lifestyle despite a relatively minor decrease in range of motion. In this case, PVNS proved to be an unlikely complication after ACL reconstruction. The patient remains at risk for the development of degenerative arthritis. [Orthopedics. 2018; 41(1):e142-e144.].

Periosteal Osteosarcoma: A Single-Institutional Study of Factors Related to Oncologic Outcomes.

BACKGROUND: Periosteal osteosarcoma is a rare surface-based variant with a lower propensity to metastasis and better prognosis than conventional osteosarcoma. The literature supporting survival benefit with adjuvant chemotherapy is lacking. Our institutional practice is for chemotherapy to be offered to patients with high-grade disease. METHODS: We conducted a retrospective cohort study of patients managed for periosteal osteosarcoma from 1970 to 2015 analyzing the survival outcomes and assessing for any relationship of survival to patient- or treatment-related factors. 18 patients were included. The study population presented at a mean of 20.8 years and was followed for a mean of 10.7 years. Factors assessed for an association with survival included age, size of tumor, use of chemotherapy, presence of medullary involvement, presence of high-grade disease, local recurrence, and site of disease. Kaplan-Meier survival analysis and Cox proportional hazard regression were performed to calculate the survival rates and to assess for the effect of any factor on survival. RESULTS: 10-year overall survival rate was 77.1%, and 10-year event-free survival rate was 66.4%. No factor was found to have an association with overall or event-free survival. CONCLUSION: These findings add to the available evidence which has failed to find any survival benefit from chemotherapy; patients with this rare disease and their families should be counselled regarding the unclear role of chemotherapy in this rare subtype of osteosarcoma.

Long-Term Functional Outcomes of Distal Femoral Replacements Compared to Geographic Resections for Parosteal Osteosarcomas of the Distal Femur.

Background: Parosteal osteosarcoma is a rare tumor with increased survival compared to conventional high-grade osteosarcoma. Due to this increased survival comes the need for reconstructive options that provide good long-term functional results. Current treatment methods include geographic resection with allograft reconstruction versus resection and reconstruction with a distal femoral replacement. Purpose: Our purpose was to compare the long-term functional outcomes of distal femoral replacements to allograft reconstructions, using the musculoskeletal tumor society (MSTS) scoring system. Methods: After querying our database, 12 patients were identified and completed a MSTS questionnaire. Results: There was no difference in functional outcomes between the cohorts at an average of 14 years follow up for the endoprosthetic group and 25 years of follow up for the geographic resection group. Conclusion: At long-term follow-up, patients who undergo a distal femoral replacement for a parosteal osteosarcoma have no difference in functional outcomes compared to those who undergo an allograft reconstruction.Level of Evidence: IV.