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.

Why Rotationplasty? A Qualitative Study of Decision-Making by Families of Patients With Primary Bone Sarcoma.

Rotationplasty is an established technique that is indicated as part of the surgical reconstruction for certain patients with primary bone tumors around the knee who undergo tumor resection. There is considerable variation in the application of rotationplasty by surgeons as well as acceptance of the procedure by patients who may be candidates for this procedure. We qualitatively studied the decision-making process of families of patients who had undergone rotationplasty by interviewing 4 patients and their families using semi-structured interviews. Thematic analysis identified the following themes that were important in the decision-making process: (1) the desire for good information sources, (2) finding value in meeting with other patients who had been faced with a similar decision, (3) prioritizing function over cosmesis, (4) a desire to limit the need for revision surgeries, and (5) accepting that a return to normalcy is not an option with a surgery. Physicians and patients faced with a similar decision can benefit from a better understanding of the process, and by the normalization of anxieties and concerns that they may experience.

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.

Reirradiation in Conservative Salvage of Recurrent Soft-tissue Sarcoma: An Analysis of Treatment Efficacy and Toxicities.

OBJECTIVE: Compared with radical resection alone, perioperative radiation therapy (RT) combined with neurovascular preserving surgery is the standard for the management of virgin soft-tissue sarcomas. Yet, the optimal management of a local recurrence remains unclear. We report outcomes of patients with locally recurrent soft-tissue sarcoma treated with resection and reirradiation at the University of Florida. MATERIALS AND METHODS: We reviewed the records of patients treated with primary conservative surgery and radiation for soft-tissue sarcoma followed by salvage resection and reirradiation for a local recurrence at our institution. RESULTS: We analyzed 23 patients treated between 1976 and 2014 (median follow-up, 46 mo). Tumor sites included: proximal extremity, 11 patients; trunk, 6; distal extremity, 5; and head and neck, 1. All patients had conservative gross total resection of their recurrent tumor, without amputation. For reirradiation, 16 patients received external-beam RT alone, 6 received external-beam RT and brachytherapy, and 1 received brachytherapy alone. Two patients received chemotherapy. After retreatment, the 5-year overall survival, cause-specific survival, local control, and distant control rates were 39%, 42%, 46%, and 60%, respectively. Ten patients experienced local recurrences, 1 experienced regional recurrence, and 9 developed distant metastases. Retreatment-related complications ranged from delayed wound healing to limb amputation; 8 patients required amputation. Only 3 patients remained disease-free at last follow-up. No statistically significant associations were found between treatment factors (eg, RT dose) and local control. CONCLUSIONS: Achieving local control of recurrent soft-tissue sarcoma is challenging. Treatment with reoperation and reirradiation can lead to debilitating complications affecting function and quality of life.

Survival and NF1 Analysis in a Cohort of Orthopedics Patients with Malignant Peripheral Nerve Sheath Tumors.

Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor syndrome in which benign plexiform neurofibromas are at risk of transforming into malignant peripheral nerve sheath tumors (MPNSTs), a very rare soft-tissue sarcoma. The prognosis of patients with MPNSTs is poor, with most studies reporting <50% survival at five years. However, studies evaluating MPNSTs are limited and report heterogeneous results. Because no MPNST-specific evidence-based treatment guideline exists, individual institutional experiences are very informative to the field. The main objective of this study was to investigate and report MPNST prognostic clinical and genetic biomarkers from our institution's Orthopedics service experience treating 20 cases from 1992 to 2017. Most patients were treated with resection and adjuvant radiation. Extended follow-up, averaging 11.4 years (ranging 1.1 to 25.1), revealed excellent five-year survival rates: 70% for overall and 60% for metastatic disease. An S100 B immunonegative tumor phenotype was associated with a significantly worse outcome than MPNSTs with positive S100 B stain. In addition, NF1 gene mutation analysis was performed on 27 families with NF1 in which at least one affected family member developed MPNSTs. Of the 27 NF1 germline mutations, five were large deletions spanning (or nearly spanning) the gene (18.5%), substantially more than such deletions in NF1 in general, consistent with increased risk of MPNSTs in such cases.

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.

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.

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.

Treatment of Sarcoma Lung Metastases with Stereotactic Body Radiotherapy.

BACKGROUND: The most common site of sarcoma metastasis is the lung. Surgical resection of pulmonary metastases and chemotherapy are treatment options that have been employed, but many patients are poor candidates for these treatments for multiple host or tumor-related reasons. In this group of patients, radiation might provide a less morbid treatment alternative. We sought to evaluate the efficacy of radiotherapy in the treatment of metastatic sarcoma to the lung. METHODS: Stereotactic body radiotherapy (SBRT) was used to treat 117 pulmonary metastases in 44 patients. Patients were followed with serial computed tomography imaging of the chest. The primary endpoint was failure of control of a pulmonary lesion as measured by continued growth. Radiation-associated complications were recorded. RESULTS: The majority of patients (84%) received a total dose of 50 Gy per metastatic nodule utilizing an image-guided SBRT technique. The median interval follow-up was 14.2 months (range 1.6-98.6 months). Overall survival was 82% at two years and 50% at five years. Of 117 metastatic nodules treated, six nodules showed failure of treatment (95% control rate). Twenty patients (27%) developed new metastatic lesions and underwent further SBRT. The side effects of SBRT included transient radiation pneumonitis (n=6), cough (n=2), rib fracture (n=1), chronic pain (n=1), dermatitis (n=1), and dyspnea (n=1). CONCLUSION: Stereotactic body radiotherapy is an effective and safe treatment for the ablation of pulmonary metastasis from sarcoma. Further work is needed to evaluate the optimal role of SBRT relative to surgery or chemotherapy for treatment of metastatic sarcoma.

The Treatment and Outcomes of Extraskeletal Osteosarcoma: Institutional Experience and Review of The Literature.

BACKGROUND: Extraskeletal osteosarcoma is a rare tumor with a poor prognosis. The purpose of this study is to examine the oncologic outcomes of this disease as they relate to surgical treatment and use of adjuvant therapies. METHODS: We retrospectively analyzed all patients treated at our institution for high-grade extraskeletal osteosarcoma of the limb or chest wall. We recorded demographic data, presenting stage, surgical margin, use of adjuvant chemotherapy or radiation, incidence of local recurrence, metastases, and death. Overall and event-free survival were calculated using Kaplan-Meier survival methods. RESULTS: There were 12 patients treated with primary wide resection or re-excision of a previously operated tumor bed. Four patients presented with metastases. Seven patients received chemotherapy and four patients received radiation therapy. There were two local recurrences, six patients developed new metastases, and nine patients died. There was no difference in overall survival in patients who received chemotherapy. There was, however, a trend towards increased length of survival in patients who received chemotherapy compared to those who did not (16.4 months vs. 9.3 months, p=0.16). CONCLUSIONS: Despite no difference in overall survival, patients treated with adjuvant chemotherapy have a trend towards increased length of survival. We suggest that extraskeletal osteosarcoma be treated with standard osteosarcoma chemotherapy regimens in addition to wide resection.

Pulmonary Surveillance Strategies Following Sarcoma Excision Vary Among Orthopedic Oncologists: A Survey of the Musculoskeletal Tumor Society.

BACKGROUND: Pulmonary surveillance protocols following sarcoma excision based on clinical evidence and outcomes are limited in current literature. The purpose of this study was to determine the method, frequency, and reasoning behind pulmonary surveillance strategies in patients treated for sarcoma among members of the Musculoskeletal Tumor Society (MSTS). METHODS: SurveyMonkey, an online survey tool, was used to create and distribute a questionnaire to 211 members of the MSTS in 2011. The 16 questions focused on current pulmonary surveillance algorithms and their reasoning. RESULTS: Of the surveyed members of the MSTS, 65% follow high-grade sarcoma with routine chest CT scans. Most disagreement involved low-grade sarcomas, where radiographs (34%), routine CT (33%), or selective CT scans (31%) were evenly distributed. Selective CT scans in low-grade lesions were warranted with an indeterminate nodule on prior CT (81%), local recurrence (40%), or large/ deep tumor characteristics (31%). Most protocols were based on continuation of training protocols (46%), clinician's interpretation of the current literature (23%), or personal experience (14%). CONCLUSIONS: Significant clinician variability exists in terms of pulmonary surveillance of sarcomas, most notably in low-grade lesions. The results of this study represent an area in need of further study to develop an evidence-based protocol for sarcoma pulmonary surveillance.

A Prospective Outcomes Study of Proton Therapy for Chordomas and Chondrosarcomas of the Spine.

PURPOSE: To evaluate the effectiveness of definitive or adjuvant external beam proton therapy on survival in patients with chordomas and chondrosarcomas of the spine. METHODS AND MATERIALS: Between March 2007 and May 2013, 51 patients with a median age of 58 years (range, 22-83 years) with chordoma (n=34) or chondrosarcomas (n=17) of the sacrum (n=21), the cervical spine (n=20), and the thoracolumbar spine (n=10) were treated with external beam proton therapy to a median dose of 70.2 Gy(RBE) [range, 64.2-75.6 Gy(RBE)] at our institution. Distant metastases, overall survival, cause-specific survival, local control, and disease-free survival were calculated. RESULTS: The mean follow-up time was 3.7 years (range, 0.3-7.7 years). Across all time points, 25 patients experienced disease recurrence: 18 local recurrences, 6 local and distant recurrences, and 1 distant metastasis. The 4-year rates of overall survival and cause-specific survival were 72%; disease-free survival was 57%, local control was 58%, and freedom from distant metastases was 86%. The median time to local progression was 1.7 years (range, 0.2-6.0 years), and the median time to distant progression was 1.6 years (range, 0.2-6.0 years). The risk factors for local recurrence were age ≤58 years (62% vs 26%; P=.04) and recurrence after prior surgery (29% vs 81%; P=.01). Secondary cancers developed in 2 patients: B-cell lymphoma 5.5 years after treatment and bladder cancer 2 years after treatment. We observed the following toxicities: sacral soft tissue necrosis requiring surgery (n=2), T1 vertebral fracture requiring fusion surgery (n=1), chronic urinary tract infections (n=1), surgery for necrotic bone cyst (n=1), and grade 2 bilateral radiation nephritis (n=1). CONCLUSION: High-dose proton therapy controls more than half of spinal chordomas and chondrosarcomas and compares favorably with historic photon data. Local progression is the dominant mode of treatment failure and may be reduced by treating patients at the time of initial diagnosis. The impact of age is a novel finding of this study.

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.

Function Preservation After Conservative Resection and Radiotherapy for Soft-tissue Sarcoma of the Distal Extremity: Utility and Application of the Toronto Extremity Salvage Score (TESS).

OBJECTIVE: To evaluate outcomes after conservative resection and radiotherapy (RT) for soft-tissue sarcoma (STS) of the distal extremity, with assessment of functional quality of life using the validated Toronto Extremity Salvage Score (TESS) questionnaire and Common Terminology Criteria for Adverse Events (CTCAE), v4.0. METHODS: Thirty-three patients with STS involving the hand/wrist (N=18) or foot/ankle (N=15) complex received adjuvant RT with conservative resection and were evaluated for local tumor control, survival, toxicities, and preservation of objective functional ability. Eight patients were treated with preoperative RT (median dose, 50.4 Gy) and 25 with postoperative RT (median dose, 61.8 Gy). Median follow-up was 11.5 years. Functional outcomes were measured using TESS; patients with amputations were excluded from the TESS analysis. Adverse events related to gait, limb edema, skin infection, wound complication, and wound dehiscence were assessed using the CTCAE. RESULTS: The 5- and 10-year local control rates were both 90%. The 10-year cause-specific, absolute, and distant metastasis-free survival rates were 97%, 87%, and 84%, respectively. Three patients had an amputation for reasons other than local recurrence or treatment complications and underwent amputation for patient preference. One third of the subjects (11/33 patients) were able to complete the TESS questionnaire; scores ranged from 88 to 100 (mean, 98.2). CTCAEv4 acute adverse events occurred in 2 cases: 1 patient had a grade 3 skin infection and 1 had a grade 2 wound complication of dehiscence. CONCLUSIONS: For management of distal extremity STS, the combination of adjuvant RT and conservative surgery achieves excellent local control and overall survival with few adverse events. In addition, through application of the TESS survey instrument, we have demonstrated that this treatment plan achieves robust functional preservation objectively and quantifiably.