Enormous myxoid/round cell liposarcoma: A case report.

Myxoid/round cell liposarcomas (MRCLPS) are a rare soft tissue sarcoma. We report the largest sarcoma in our institutional history. We discuss the patient's surgical management and treatment of the tumor and challenges given its dimensions. Several complications arose following primary resection that were managed by a multidisciplinary team. Although MRCLPS can vary in size, large MRCLPS must be treated cautiously given the potential for complications. Additionally, multidisciplinary treatment of MRCLPS is essential in diagnosing and treating these complex cases.

Total Humeral Endoprosthetic Reconstruction: A Systematic Review.

BACKGROUND: Total humeral endoprosthetic reconstruction (THER) is a rare reconstruction option for limb salvage surgery for large humeral neoplasms or bone destruction. MATERIALS AND METHODS: Because of the limited data and need for this procedure, we reviewed the literature surrounding THER and assessed functionality, complications, and revisions using the PubMed, Embase, Ovid, and Scopus databases. RESULTS: Among 29 articles and 175 patients, the most common indication was neoplasm (n=25, 86%), mean follow-up was 61.98 months (SD=55.25 months), and mean Musculoskeletal Tumor Society score was 73.64% (SD=10.69%). Reported complications included 26 (23%) revisions in 7 studies, 35 (36%) cases of shoulder instability in 7 studies, and 13 (13.54%) cases of deep infection in 4 studies. CONCLUSION: THER should be considered with a thorough knowledge of outcomes and potential complications to guide patient and clinician expectations. [Orthopedics. 2024;47(3):e106-e113.].

Myoepithelial Carcinoma of Soft Tissue: Report of Two Cases.

CASE: A 40-year-old man was evaluated for a painful mass on his right calf, and a 36-year-old woman presented with a painless mass on her right foot. Final pathology revealed marked nuclear atypia and positivity for S100/SOX10 and AE1/AE3 confirming diagnoses of myoepithelial carcinoma. Both patients underwent surgical resection and are without evidence of local recurrence or metastatic disease at 1-year follow-up. CONCLUSION: Soft-tissue tumors presenting in the extremities warrant careful evaluation and timely histopathologic diagnosis. Myoepithelial carcinomas are rare, aggressive tumors with a propensity for local recurrence and metastasis. Treatment of these tumors should be discussed by a multidisciplinary tumor team.

Development and external validation of a machine learning model for prediction of survival in extremity leiomyosarcoma.

PURPOSE: Machine learning (ML) models have been used to predict cancer survival in several sarcoma subtypes. However, none have investigated extremity leiomyosarcoma (LMS). ML is a powerful tool that has the potential to better prognosticate extremity LMS. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried for cases of histologic extremity LMS (n = 634). Patient, tumor, and treatment characteristics were recorded, and ML models were developed to predict 1-, 3-, and 5-year survival. The best performing ML model was externally validated using an institutional cohort of extremity LMS patients (n = 46). RESULTS: All ML models performed best at the 1-year time point and worst at the 5-year time point. On internal validation within the SEER cohort, the best models had c-statistics of 0.75-0.76 at the 5-year time point. The Random Forest (RF) model was the best performing model and used for external validation. This model also performed best at 1-year and worst at 5-year on external validation with c-statistics of 0.90 and 0.87, respectively. The RF model was well calibrated on external validation. This model has been made publicly available at https://rachar.shinyapps.io/lms_app/ CONCLUSIONS: ML models had excellent performance for survival prediction of extremity LMS. Future studies incorporating a larger institutional cohort may be needed to further validate the ML model for LMS prognostication.

Hip Arthroplasty Outcomes in Patients with Metastatic Bone Disease.

Background/Objective: The hip is a common location for metastatic bone disease (MBD) and surgical intervention is often indicated to relieve pain and improve function. MBD of the hip is usually treated with hemiarthroplasty or with total hip arthroplasty if there are acetabular lesions. As treatment for metastatic disease evolves and patients may expect to live longer after diagnosis, further evaluation of the complications and functional outcomes associated with hip arthroplasty for MBD are necessary. Methods: This was a retrospective review of patients who underwent hip arthroplasty for MBD at a single institution between 2007 and 2021. Outcomes included rates of reoperation, complications, and overall survival. Results: Ninety-three cases in 91 patients were included. Total hip arthroplasty (THA) was performed in 52 cases (55.9%), hemiarthroplasty in 15 (16.1%), and complex arthroplasty, including proximal femur replacement or THA with complex acetabular reconstruction, was performed in 26 (28%). There were 11 reoperations in five patients and six dislocations. Median survival was 10.4 months and one-year overall survival was 53.3%. There was a significant increase in the proportion of patients who were able to ambulate independently (35.2% vs 17.6%; p=0.02) and a significant decrease in patients confined to a wheelchair (18.7% vs 3.3%; p=0.045) after surgery. Conclusions: Hip arthroplasty for MBD leads to relatively low rates of complications and reoperations in this population known to have high postoperative morbidity and mortality. Hip arthroplasty provides the majority of MBD patients with a durable reconstruction that exceeds their lifespan and enables them to return to an acceptable level of function.

Intra-Articular Nodular Fasciitis of the Knee, A Rare Clinical Presentation and Histological Diagnosis: A Case Report.

Introduction: Nodular fasciitis is a rare, benign soft tissue lesion that can prove to be difficult to diagnose, especially when intra-articular. Case presentation: A 17-year-old female softball player presents with several year history of right knee pain and swelling initially attributed to patellar maltracking refractory to non-operative and operative management. Management and Outcomes: Initial pathology suggested tenosynovial giant cell tumor; however, further tissue diagnosis revealed nodular fasciitis, which was eventually resected. Conclusion: Intra-articular nodular fasciitis of the knee is rare and may easily be misdiagnosed due to its nonspecific clinical presentation. Careful histological examination can aid in diagnosis. Nodular fasciitis should be considered in the differential diagnoses for intra-articular lesions of the knee joint.

Evaluation of Absolute Neutrophil Count in the Perioperative Setting of Sarcoma Resection.

Introduction: Limb salvage surgery (LSS) is the preferred surgical treatment for bone sarcomas. Preoperatively, many patients receive chemotherapy and may develop neutropenia. No study has evaluated the effect of a low preoperative absolute neutrophil count (ANC) on postoperative outcomes following LSS. Methods: This was a retrospective review of 114 patients who underwent LSS for bone sarcoma from 2010 to 2020. Preoperative lab values were analyzed by logistic regression to identify the risk of developing surgical complications within 30 days, surgical site infection (SSI), and reoperation. Results: Three (2.6%) patients experienced a surgical complication within 30 days. Twelve (10.53%) patients experienced postoperative SSI. Twenty-nine (25.4%) required reoperation. Preoperative ANC was not a significant predictor of surgical complications within 30 days, SSI, or reoperation. The only independent predictor of worse overall survival was the presence of a pathologic fracture at the time of surgery. Conclusion: This is the first study to evaluate preoperative ANC on postoperative outcomes following LSS. We report no significant differences in surgical complications within 30 days, SSI, or reoperation with low preoperative ANC. Future studies with larger cohorts of neutropenic patients are needed to evaluate these outcomes, as our cohort had very few neutropenic patients due to selection bias.

Internal hemipelvectomy: A single institution's learning curve and longitudinal experience.

BACKGROUND AND OBJECTIVES: Wide margin resection for pelvic tumors via internal hemipelvectomy is among the most technically challenging procedures in orthopedic oncology. As such, surgeon experience and technique invariably affect patient outcomes. The aim of this clinical study was to assess how an individual surgeon's experiences and advancements in technology and techniques in the treatment of internal hemipelvectomy have impacted patient outcomes at our institution. METHODS: This study retrospectively examined a single tertiary academic institution's consecutive longitudinal experience with internal hemipelvectomy for primary sarcoma or pelvic metastases over a 26-year period between the years 1994 and 2020. Outcomes were assessed using two separate techniques. The first stratified patients into cohorts based on the date of surgery with three distinct "eras" ("early," "middle," and "modern"), which reflect the implementation of new techniques, including three-dimensional (3D) computer navigation and cutting guide technology into our clinical practice. The second method of cohort selection grouped patients based on each surgeon's case experience with internal hemipelvectomy ("inexperienced," "developing," and "experienced"). Primary endpoints included margin status, complication profiles, and long-term oncologic outcomes. Whole group multivariate analysis was used to evaluate variables predicting blood loss, operative time, tumor-free survival, and mortality. RESULTS: A total of 72 patients who underwent internal hemipelvectomy were identified. Of these patients, 24 had surgery between 1994 and 2007 (early), 28 between 2007 and 2015 (middle), and 20 between 2016 and 2020 (modern). Twenty-eight patients had surgery while the surgeon was still inexperienced, 24 while developing, and 20 when experienced. Evaluation by era demonstrated that a greater proportion of patients were indicated for surgery for oligometastatic disease in the modern era (0% vs. 14.3% vs. 35%, p = 0.022). Fewer modern cases utilized freehand resection (100% vs. 75% vs. 55%, p = 0.012), while instead opting for more frequent utilization of computer navigation (0% vs. 25% vs. 20%, p = 0.012), and customized 3D-printed cutting guides (0% vs. 0% vs. 25%, p = 0.002). Similarly, there was a decline in the rate of massive blood loss observed (72.2% vs. 30.8% vs. 35%, p = 0.016), and interdisciplinary collaboration with a general surgeon for pelvic dissection became more common (4.2% vs. 32.1% vs. 85%, p < 0.001). Local recurrence was less prevalent in patients treated in middle and modern eras (50% vs. 15.4% vs. 25%, p = 0.045). When stratifying by case experience, surgeries performed by experienced surgeons were less frequently complicated by massive blood loss (66.7% vs. 40% vs. 20%, p = 0.007) and more often involved a general surgeon for pelvic dissection (17.9% vs. 37.5% vs. 65%, p = 0.004). Whole group multivariate analysis demonstrated that the use of patient-specific instrumentation (PSI) predicted lower intraoperative blood loss (p = 0.040). However, surgeon experience had no significant effect on operative time (p = 0.125), tumor-free survival (p = 0.501), or overall patient survival (p = 0.735). CONCLUSION: While our institution continues to utilize neoadjuvant and adjuvant therapies following current guideline-based care, we have noticed changing trends from early to modern periods. With the advent of new technologies, we have seen a decline in freehand resections for hemipelvectomy procedures, and a transition to utilizing more 3D navigation and customized 3D cutting guides. Furthermore, we have employed the use of an interdisciplinary team approach more regularly for these complicated cases. Although our results do not demonstrate a significant change in perioperative outcomes over the years, our institution's willingness to treat more complex cases likely obscures the benefits of surgeon experience and recent technological advances for patient outcomes.

Does Perioperative Radiation Affect Implant Survivorship of Primary Total Hip Arthroplasty in the Setting of Metastatic Bone Disease?

Background: Metastatic bone disease (MBD) commonly affects the hip and surgical intervention including total hip arthroplasty (THA) is often indicated to treat the joint and improve function. Patients with metastatic cancer often receive radiotherapy, and orthopaedic oncologists must consider surgical risks with operating on irradiated bone and soft tissue. We evaluated surgical outcomes and implant survival (IS) of titanium acetabular components and femoral components in patients treated for MBD in the setting of perioperative radiation. Methods: This was a retrospective review of patients who underwent THA for MBD at 3 institutions between 2017 and 2021. Outcomes included rates of reoperation, complications, IS, and overall survival. Results: Forty-six patients who received primary THA for MBD were included in the study. Twenty patients (43.5%) received perioperative radiation for MBD. Six postoperative complications including one superficial wound infection, 2 dislocations, 2 pathologic fractures, and one aseptic acetabular component loosening led to 5 reoperations. There were no significant differences in postoperative outcomes, reoperation after THA, and IS based on radiotherapy status. Conclusions: To our knowledge, this is the first paper evaluating primary THA outcomes and IS between patients who receive perioperative radiation for MBD to the hip and those who do not. As surgical management is a crucial part of the treatment in alleviating pain and disability in patients with MBD, we continue to recommend THA for patients who received radiation at the operative site.

An Unusual Case of Clear Cell Chondrosarcoma With Early Metastatic Recurrence.

We present a case of a 58-year-old male who presented following 4 months of progressively worsening right upper extremity pain. Initial pathology demonstrated pleomorphic chondroblasts with increased mitotic activity indicating an intermediate grade (Grade 2) clear cell chondrosarcoma of the proximal humerus. Following surgical resection, the primary lesion demonstrated aggressive behavior and early metastasis to the cervical and thoracic spine. The patient unfortunately expired 30 months after initial presentation. Although clear cell chondrosarcoma has been historically regarded as low grade, we present a unique example of an aggressive clinical course of clear cell chondrosarcoma.

Rare Dedifferentiated Periosteal Chondrosarcoma with 11-Year Metastatic Relapse: A Case Report.

A 50-year-old man presented for evaluation of a periscapular mass. Biopsy suggested a low-grade chondrosarcoma; however, the resected specimen revealed a grade 2 chondrosarcoma with a low-grade dedifferentiated mesenchymal component. The mass multiply recurred as chondrosarcoma without a dedifferentiated component before the patient developed lung metastases of chondrosarcoma without a dedifferentiated component 11 years after the initial diagnosis and died of disease. This is one of the first reported cases of a dedifferentiated chondrosarcoma with low-grade dedifferentiated component. While overall prognosis may be better than in typical dedifferentiated chondrosarcoma, this tumor demonstrated numerous local recurrences as well as metastasis.

The effect of timing between preoperative embolization and surgery: A retrospective analysis of hypervascular bone metastases.

BACKGROUND AND OBJECTIVES: The optimal timing between preoperative embolization of hypervascular metastatic bone lesions and surgery has yet to be established. Our analysis sought to evaluate embolization timing impacts blood loss, transfusion risk, and operative time in patients with hypervascular primary tumors. METHODS: We identified patients with renal cell (RCC) or thyroid carcinoma undergoing surgery between 1992 and 2023. Patients were segregated into the following cohorts: (1) no embolization preoperatively, (2) surgery <24 h of embolization, and (3) surgery >24 h after embolization. Multivariate logistic regression analyses were performed to assess the effect of embolization timing while controlling for confounding variables. RESULTS: No differences were seen in all evaluated outcomes between immediate and delayed embolization cohorts. No differences in estimated blood loss were seen between the immediate (OR: 0.685, 95% CI: 0.159-2.949; p = 0.611) and delayed (OR: 0.568, 95% CI: 0.093-3.462; p = 0.539) surgery cohorts compared with patients without embolization. Surgery >24 h after embolization was not associated with a higher risk of prolonged operative time (OR: 13.499, 95% CI: 0.832-219.146; p = 0.067). CONCLUSIONS: These findings suggest that surgery may be safely delayed beyond 24 h from embolization without a higher risk of bleeding. In appropriately selected cohorts, embolization may not be needed preoperatively.

Challenges in the Management of Complex Soft-Tissue Sarcoma Clinical Scenarios.

With nearly 15,000 new cases of soft-tissue sarcoma (STS) in the United States each year, early diagnosis and therapeutic management is imperative for successful patient outcomes. Primary STS is conventionally treated with a combination of wide-margin resection, neoadjuvant or adjuvant radiation therapy, and, in specific cases, adjuvant chemotherapy. However, in situations of complex disease presentation, guidelines for treatment are less clearly outlined. Limited metastatic disease, local recurrence, fungating STSs, and unplanned or incomplete resections of STSs present unique challenges for the multidisciplinary care team. The management of complex STS clinical scenarios usually involves limb-salvage resection or amputation as well as some combination of radiation therapy and/or ablative interventional radiology techniques. As a result, a multidisciplinary team approach is essential for treating patients in these challenging scenarios, with a recent focus on the integration of plastic and reconstructive surgery into the treatment algorithm.

Opiate use after total hip arthroplasty for metastatic bone disease.

OBJECTIVES: To investigate post-operative opioid use following a total hip arthroplasty (THA) in metastatic bone disease (MBD) patients and identify factors associated with post-operative opioid use at 6 weeks and 90 days. BACKGROUND: MBD commonly affects the hip, and surgical intervention including THA may be indicated for pain relief or to improve function. Following THA, patients are often prescribed short courses of opioids for post-operative pain relief. No study has evaluated opiate use following THA in patients for MBD. METHODS: This was a retrospective review of patients using opioids preoperatively who underwent primary THA for MBD at two institutions between 2009 and 2022. Preoperative and post-operative opioid usages, respectively, at 6 weeks and 90 days were quantified through calculating daily morphine milligram equivalents (MMEs) and compared using the sign test. Factors associated with post-operative opioid use at 6 weeks and 90 days were compared using χ2 test or Fisher's exact test as appropriate. RESULTS: Nineteen THA and 11 THA with complex acetabular reconstruction were included. At 6 weeks, 26 (86.7 percent) patients were utilizing opiates, and at 90 days, 23 (76.7 percent) patients were utilizing opiates. There was a statistically significant difference between median daily preoperative MME compared to daily MME at 90 days (p < 0.001). The only statistically significant association with opioid use at 90 days was opioid use at 6 weeks. CONCLUSION: To our knowledge, this is the first paper evaluating post-operative opioid use following primary THA in MBD patients. After THA in the setting of MBD, patients exhibit decreased post-operative opioid use. Future studies with larger cohorts should be conducted to characterize post-operative opioid use following joint arthroplasty in MBD patients.

Clinical Outcomes Following Resection of Distal Lower Extremity Soft Tissue Sarcomas.

OBJECTIVE: Resection of soft tissue sarcoma (STS) in the distal lower extremity can result in large soft tissue defects that create difficult wounds to manage and significant risks for complications. Often, the anticipated or resultant tissue defect or loss of functional anatomy is an indication for amputation over limb salvage. Soft tissue reconstruction managed by plastic and reconstructive surgery (PRS) may help offer limb salvage as a therapeutic option with acceptable oncologic outcomes and wound complication rates. METHODS: This was a review of 52 patients who underwent resection of STS at the level of the knee or distal between 2010 and 2020. Plastic and reconstructive surgery soft tissue management was utilized in 40.4% (n = 21) of cases, most of whom would have otherwise been considered candidates for amputation. RESULTS: The overall rate of limb salvage was 76.9%. The overall rate of wound complications was 19.2%. The overall rate of negative margins of resection was 92.3%. The 1-, 5-, and 10-year overall survival probabilities were 92%, 85%, and 85%, respectively. Of the 40 limb salvage procedures, two required subsequent amputation, one for multiply recurrent disease and one for necrosis. The wound complication rate was 14.3% in the PRS management group and 22.6% in cases of wound closure managed by the primary surgeon. There were zero instances of total wound or flap loss in PRS- managed closures. When comparing patients with wound complications to those without, there was no difference in age (59.5 ± 21 vs. 51 ± 18 years, p = 0.42), body mass index (31.1 ± 4.8 vs. 26.1 ± 7.1 kg/m2 , p = 0.19), or tumor size (6.8 ± 5.0 vs. 6.4 ± 4.7 cm, p = 0.82). At final follow-up, 67% (n = 35) of patients were alive and disease-free. CONCLUSIONS: Wound complications are not uncommon after resection of distal lower extremity STS. Our experience has been successful in achieving limb salvage in these challenging cases. Without PRS soft tissue management, however, many patients may alternatively be candidates for amputation.

Radiolucent implants in orthopedic oncology.

Radiolucent implants in have demonstrated promising results for both extremity and spine oncologic procedures. However, questions persist about whether the superiority in surveillance imaging justify the increased cost and technical challenges. In this review, we present the current body of literature for the use of radiolucent implants in musculoskeletal oncology, with a focus on implant complications, including screw loosening, breakage, malposition, and loss of reduction. We also discuss clinical outcomes, technical considerations, and postoperative radiotherapy.

Patient-reported outcome tools in musculoskeletal oncology.

Patient-reported outcomes (PRO) are collected directly from the patient and have become increasingly utilized in the clinical setting and in clinical research. In musculoskeletal oncology patients, a number of patient-reported outcomes measures (PROM) have been developed and investigated to evaluate functional outcomes and health-related quality of life in these patients. With the growing evidence for PROM in musculoskeletal oncology patients, PROM should be considered for the clinical care of these patients.

A Rare Location of Osteosarcoma in the Cuboid Bone: A Case Report.

CASE: In this case report, we describe a patient with left foot pain whose magnetic resonance imaging revealed a destructive tumor of the left cuboid bone. A biopsy of the tumor confirmed an osteoblastic osteosarcoma of the cuboid, and the patient was treated with chemotherapy followed by a below-the-knee amputation. CONCLUSION: There are only a handful of cases reporting osteosarcoma of the cuboid bone, and challenges remain in properly diagnosing the lesion to recommend the best course of treatment.

The First Cold Atmospheric Plasma Phase I Clinical Trial for the Treatment of Advanced Solid Tumors: A Novel Treatment Arm for Cancer.

Local regional recurrence (LRR) remains the primary cause of treatment failure in solid tumors despite advancements in cancer therapies. Canady Helios Cold Plasma (CHCP) is a novel Cold Atmospheric Plasma device that generates an Electromagnetic Field and Reactive Oxygen and Nitrogen Species to induce cancer cell death. In the first FDA-approved Phase I trial (March 2020-April 2021), 20 patients with stage IV or recurrent solid tumors underwent surgical resection combined with intra-operative CHCP treatment. Safety was the primary endpoint; secondary endpoints were non-LRR, survival, cancer cell death, and the preservation of surrounding healthy tissue. CHCP did not impact intraoperative physiological data (p > 0.05) or cause any related adverse events. Overall response rates at 26 months for R0 and R0 with microscopic positive margin (R0-MPM) patients were 69% (95% CI, 19-40%) and 100% (95% CI, 100-100.0%), respectively. Survival rates for R0 (n = 7), R0-MPM (n = 5), R1 (n = 6), and R2 (n = 2) patients at 28 months were 86%, 40%, 67%, and 0%, respectively. The cumulative overall survival rate was 24% at 31 months (n = 20, 95% CI, 5.3-100.0). CHCP treatment combined with surgery is safe, selective towards cancer, and demonstrates exceptional LRR control in R0 and R0-MPM patients. (Clinical Trials identifier: NCT04267575).

Association between neutrophil-lymphocyte ratio, platelet-lymphocyte ratio, and survival in undifferentiated pleomorphic sarcoma (NLR, PLR, and overall survival in UPS).

BACKGROUND AND OBJECTIVES: Cancer-related inflammation has been shown to be a driver of tumor growth and progression, and there has been a recent focus on identifying markers of the inflammatory tumor microenvironment. Neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) are inflammatory indices that have been identified as prognostic biomarkers in various malignancies. However, there is limited and conflicting data regarding their prognostic value in soft tissue sarcoma (STS) and specifically in undifferentiated pleomorphic sarcoma (UPS). METHODS: This was a retrospective review of patients who underwent surgical treatment for primary UPS from 1993 to 2021. Cutoff values for NLR and PLR were determined by receiver operating curve analysis. Cox proportional hazards regression was used to determine prognostic factors on univariate and multivariate analysis. RESULTS: Eighty-six patients were included. The optimal cutoff value was 3.3 for NLR and 190 for PLR. Both high NLR (HR 2.44; 95% CI 1.29-4.63; p = 0.005) and high PLR (HR 1.99; 95% CI 1.08-3.67, p = 0.02) were associated with worse OS on univariate analysis. On multivariate analysis, metastasis at presentation and radiotherapy were independently predictive of OS, but high NLR (HR 1.30; 95% CI 0.64-2.98; p = 0.41) and high PLR (HR 1.63; 95% CI 0.82-3.25; p = 0.17) were not predictive of survival. CONCLUSIONS: High pre-treatment NLR and PLR were associated with decreased overall survival but were not independent predictors of survival in patients undergoing resection for UPS. Until additional prospective studies can be done, survival outcomes are best predicted using previously established patient- and tumor-specific factors.