Does adding sacroiliac (type IV) resection to periacetabular (type II) resection increase complications or provide worse clinical outcomes? An institutional experience and systematic review.

BACKGROUND AND OBJECTIVES: Internal hemipelvectomy is a limb sparing procedure most commonly indicated for malignant bone and soft tissue tumors of the pelvis. Partial resection and pelvic reconstruction may be challenging for orthopedic oncologists due to late presentation, high tumor burden, and complex anatomy. Specifically, wide resection of tumors involving the periacetabular and sacroiliac (SI) regions may compromise adjacent vital neurovascular structures, impair wound healing, or limit functional recovery. We aimed to present a series of patients treated at our institution who underwent periacetabular internal hemipelvectomy (Type II) with or without sacral extension (Type IV) in combination with a systematic review to investigate postoperative complications, functional outcomes, and implant and patient survival following pelvic tumor resection via Type II hemipelvectomy with or without Type IV resection. MATERIALS AND METHODS: A surgical registry of consecutive patients treated with internal hemipelvectomy for primary or secondary pelvic bone tumors at our institution since 1994 was retrospectively reviewed. All type II resection patients were stratified into two separate cohorts, based on whether or not periacetabular resection was extended beyond the SI joint to include the sacrum (Type IV), as per the Enneking and Dunham classification. Patient demographics, operative parameters, complications, and oncological outcomes were collected. Categorical and continuous variables were compared with Pearson's chi square or Fisher's exact test and the Mann-Whitney U test, respectively. Literature review according to PRISMA guidelines queried studies pertaining to patient outcomes following periacetabular internal hemipelvectomy. The search strategy included combinations of the key words "internal hemipelvectomy", "pelvic reconstruction", "pelvic tumor", and "limb salvage". Pooled data was compared using Pearson's chi square. Statistical significance was established as p < 0.05. RESULTS: A total of 76 patients were treated at our institution with internal hemipelvectomy for pelvic tumor resection, of whom 21 had periacetabular resection. Fifteen patients underwent Type II resection without Type IV involvement, whereas six patients had combined Type II/IV resection. There were no significant differences between groups in operative time, blood loss, complications, local recurrence, postoperative metastasis, or disease mortality. Systematic review yielded 69 studies comprising 929 patients who underwent internal hemipelvectomy with acetabular resection. Of these, 906 (97.5 %) had only Type II resection while 23 (2.5 %) had concomitant Type II/IV resection. While overall complication rates were comparable, Type II resection alone produced significantly fewer neurological complications when compared to Type II resection with sacral extension (3.9 % vs. 17.4 %, p = 0.001). No significant differences were found between rates of wound complications, infections, or construct failures. Local recurrence, postoperative metastasis, and survival outcomes were similar. Type II internal hemipelvectomy without Type IV resection on average produced higher postoperative MSTS functional scores than with Type IV resection. CONCLUSION: In our series, the two groups exhibited no differences. From the systematic review, operative parameters, local recurrence or systemic metastasis, implant survival, and disease mortality were comparable in patients undergoing Type II internal hemipelvectomy alone compared to patients undergoing some combination of Type II/IV resection. However, compound resections increased the risk of neurological complications and experienced poorer MSTS functional scores.

A Comparison of Surgical Approaches for Hip Hemiarthroplasty Performed for the Treatment of Femoral Neck Fracture: A Systematic Review and Network Meta-analysis of Randomized Controlled Trials.

BACKGROUND: Multiple hemiarthroplasty (HA) approaches exist for femoral neck fracture (FNF) treatment. However, there is no consensus on the superiority of one approach for postoperative outcomes. This study assessed outcomes among randomized controlled trials (RCTs) comparing the posterior approach (PA), direct lateral approach (DLA), anterolateral approach (ALA), and the direct anterior approach (DAA) in HA for FNF. METHODS: PubMed, Ovid/MEDLINE, Scopus, Cochrane Central Registry of Controlled Trials, and Cochrane Database of Systematic Reviews were queried in February 2024. A frequentist model network meta-analysis of eligible prospective RCTs compared outcomes among approaches using P-scores. RESULTS: Of 1,481 retrieved studies, 11 RCTs totaling 1,513 FNF patients who underwent HA through a PA (n = 446; 29.5%), DLA (n = 481; 31.8%), ALA (n = 296; 19.6%), or DAA (n = 290; 19.2%) with median (interquartile range) follow-up of 6 (4.5-12.0) months were included for meta-analysis. DAA was associated with statistically increased operative duration (mean difference [MD], 1.89 minutes; 95% CI, 0.08 to 3.69 minutes; p < 0.001) compared with a PA, whereas an ALA was associated with statistically greater blood loss compared with a PA (MD, 5.81 mL; 95% CI, 4.11 to 7.50 mL; p < 0.001). There were no differences in the incidence of fracture, dislocation, infection, complications, revision, nor 1-year mortality among approaches. There were also no differences in hip nor pain score improvement at latest follow-up, although with high heterogeneity (I2 = 85.7% and 94.2%, respectively). CONCLUSION: This study found no clinical difference in improvement in pain, function, nor in revision outcomes and operative characteristics among HA approaches for FNF, and each approach had a similar adverse event profile. Despite limited randomized evidence, these findings suggest comparable short-term efficacy and safety of all approaches. LEVEL OF EVIDENCE: Therapeutic, Level I. See Instructions for Authors for a complete description of levels of evidence.

Bilateral Knee Arthrodesis and Total Hip Arthroplasty for Knee and Hip Dysplasia Secondary to Spina Bifida: A Case Report.

Introduction: Spina bifida is a rare neurological condition that, if uncorrected, may cause chronic valgus knee stress difficulty with independent ambulation and significant pain. Current literature lacks comprehensive guidance on surgically correcting progressive hip and knee deformities in spina bifida patients, which include osteotomy and primary. When these options are contraindicated, alternative approaches such as arthrodesis may be indicated, although they remain understudied in this population. Case Report: A 47-year-old man presented with bilateral hip and knee pain. Radiographs demonstrated valgus knee deformities with severe arthrosis and bilateral femoral head subluxation. The patient had a history of spina bifida with chronic lower extremity weakness and neurogenic bladder. He underwent staged bilateral instrumented knee arthrodesis and staged bilateral total hip arthroplasty (THA). Conclusion: This is the first reported case of bilateral instrumented knee arthrodesis in a patient with spina bifida. Orthopedic care of these patients is complex. Surgical correction of symptomatic knee and hip deformities through instrumented arthrodesis and THA may permit an improved functional outcome.

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.

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.

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.

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.

Hypoalbuminemia Increases Mortality after Two-Stage Revision Total Joint Arthroplasty.

Objectives: This study aimed to evaluate the effect of hypoalbuminemia on failure rates and mortality after a two-stage revision for PJI. Methods: 199 Patients (130 knees and 69 hips) with a mean age of 64.7 ± 10.7 years who underwent a two-stage exchange were retrospectively reviewed at a mean of 51.2 ± 39.7 months. Failure of treatment was defined as any revision within the follow-up period, failure to undergo reimplantation, or death within one year of initiating treatment. Results: There were 71 failures (35.7%), including 38 septic failures (19.1%). We found no differences between successful revisions and failures regarding hypoalbuminemia (43% vs. 42% prior to stage 1, P=1 and 32% vs. 29% prior to stage 2, P=0.856). There were also no differences in hypoalbuminemia rates between septic failures and the rest of the cohort (42% vs. 43% prior to stage 1, P=1.0 and 34% vs. 30% prior to stage 2, P=0.674). Hypoalbuminemia prior to stage 2 was a significant predictor of mortality based on multivariate analysis (odds ratio 5.40, CI 1.19-24.54, P=0.029). Hypoalbuminemia was independently associated with a greater length of stay by 2.2 days after stage 1 (P=0.002) and by 1.0 days after the second stage reimplantation (P=0.004). Conclusion: Preoperative hypoalbuminemia is a significant predictor of mortality and increased length of stay following two-stage revision but is not a predictor of failure of PJI treatment. Further study is required to understand if hypoalbuminemia is a modifiable risk factor or a marker for poor outcomes.

A Systematic Review and Meta-Analysis of Negative Wound Pressure Therapy Use in Soft Tissue Sarcoma Resection.

Background: Negative wound pressure therapy (NWPT) may reduce the wound complication (WC) risk in soft tissue sarcoma (STS) and is often utilized for large and/or irradiated wounds, extensive dissections, or wounds at risk of impaired drainage. However, data on WCs after NWPT in STS are lacking. This study systematically reviewed the available literature on NWPT in STS. Methods: A query of the Cochrane Central Register of Controlled Trials (1976-2022), Cochrane Database of Systematic Reviews, MEDLINE (1946-2022), Scopus, and PubMed (1964-2022) was performed. Eight studies met inclusion. Results: One-hundred eighty-six cases were analyzed. Among studies with available data, myxofibrosarcoma (n=32/131; 24.4%) and undifferentiated pleomorphic sarcoma (n=29/131; 22.1%) were the most common subtypes, 83.3% (n=90/108) were lower extremity STS, and 51.9% (n=82/158) were preoperatively irradiated. The overall WC rate was 10.8% (n=20/186). Pooled-analysis (three studies) demonstrated a lower WC risk with NWPT versus conventional dressings (OR, 0.133; 95% CI, 0.050-0.351; p<0.001; I2=0%). Subsequent analysis (two studies) found no increased local recurrence risk versus conventional dressings (OR, 1.019; 95% CI, 0.125-8.321; p=0.99), with high heterogeneity. Conclusion: NWPT appears to lower the WC risk in STS without increasing the recurrence risk, and may be suitable for primary, recurrent, or positive margin resections, staged reconstructions, and while awaiting histologic margin assessment. However, larger, randomized-controlled trials of NWPT in STS are warranted. Level of Evidence: III.

Neoadjuvant radiation influences the pseudocapsule in soft tissue sarcoma: A histopathologic and radiographic evaluation.

BACKGROUND AND OBJECTIVES: A pseudocapsule surrounds soft tissue sarcoma (STS). Its composition, response to neoadjuvant radiation, and clinical significance are poorly understood. METHODS: Seventeen cases of high-grade undifferentiated pleomorphic sarcoma (UPS) were reviewed, ten of which were treated with neoadjuvant radiation. Magnetic resonance imaging (MRI) studies, pathology slides, and patient records were reviewed. RESULTS: Irradiated pseudocapsules were well-demarcated with fewer viable tumor cells and were thicker on both pathology and MRI measurements when compared to non-irradiated pseudocapsules (p < 0.001, p = 0.04, respectively). Pseudocapsule mean pathology width (MPW) was positively correlated with tumor necrosis percentage (p = 0.044), and negatively correlated with mitotic rate (p = 0.043), though pseudocapsule width measured on MRI did not correlate with these prognostic factors. Despite an evident treatment response to neoadjuvant radiation, viable tumor cells were present within the pseudocapsule and the surrounding healthy tissue. CONCLUSIONS: The pseudocapsule in STS responds to radiation and there appears to be a correlation between pseudocapsule width and tumor necrosis and mitotic activity. As viable tumor cells are present beyond the pseudocapsule, surgeons should remain cautious in determining margins of resection in STS when using the pseudocapsule as a palpable landmark. This novel study is the most detailed to date to describe the histopathologic and radiographic characteristics of the STS pseudocapsule. Further studies are needed to determine the clinical significance of the pseudocapsule.

Extraskeletal myxoid chondrosarcoma: Clinicopathological features and outcomes from the United States sarcoma collaborative database.

BACKGROUNDS AND OBJECTIVES: This investigation described clinicopathological features and outcomes of extraskeletal myxoid chondrosarcoma (EMC) patients. METHODS: EMC patients were identified from the United States Sarcoma Collaborative database between 2000 and 2016. Overall survival (OS) and recurrence-free survival (RFS) were calculated, and prognostic factors were analyzed. RESULTS: Sixty individuals with a mean age of 55 years were included, and 65.0% (n = 39) were male. 73.3% (n = 44) had a primary tumor. A total of 41.6% (n = 25) developed tumor relapse following resection. The locoregional recurrence rate was 30.0% (n = 18/60), and mean follow-up was 42.7 months. The 5-year OS was 71.0%, while the 5-year RFS was 41.4%. On multivariate analysis for all EMC, chemotherapy (hazard ratio [HR], 6.054; 95% confidence interval [CI], 1.33-27.7; p = 0.020) and radiation (HR, 5.07, 95% CI, 1.3-20.1; p = 0.021) were independently predictive of a worse RFS. Among patients with primary EMC only, the 5-year OS was 85.3%, with a 30.0% (n = 12) locoregional recurrence rate, though no significant prognostic factors were identified. CONCLUSIONS: Long-term survival with EMC is probable, however there exists a high incidence of locoregional recurrence. While chemotherapy and radiation were associated with a worse RFS, these findings were likely confounded by recurrent disease as significance was lost in the primary EMC-only subset.

En Bloc Resection of Giant Cell Tumor following Neoadjuvant Denosumab: A Case Report and Review of the Literature.

Introduction: The RANK ligand inhibitor denosumab has been used to treat cases of unresectable giant cell tumors of bone (GCTB) or preoperatively to facilitate intralesional curettage. However, there are no clear guidelines for use of denosumab prior to en bloc resection. Case Presentation: In this study, a 26-year-old patient presented with atraumatic lateral knee pain. X-rays demonstrated a destructive lesion within the proximal fibula, and biopsy confirmed the diagnosis of GCTB. Management and Outcomes: The patient received 3 months of neoadjuvant denosumab with complete resolution of his pain and cortical rim formation surrounding the tumor. The tumor was removed en bloc with negative margins. At 6-month follow-up, the patient had returned to his functional baseline with no evidence of tumor recurrence. Conclusion: Neoadjuvant denosumab can mitigate symptoms related to GCTB and promotes cortical bone formation, facilitating en bloc resection and permitting acceptable functional outcomes in select cases.

Dedifferentiated Chondrosarcoma: A Case Series and Review of the Literature.

Background: Dedifferentiated chondrosarcoma (DCS) is a rare and aggressive malignancy with a poor prognosis. The purpose of this investigation was to describe our treatment and outcomes of 16 DCS patients at our institution and provide a review of the current literature. Methods: This study was a retrospective review over a consecutive twenty-year period. Data including treatment details and outcomes were recorded. Results: A total of 16 cases from 2000 to 2018 were identified. The median age (IQR) was 62 years (52; 69) and the majority of DCS arose in the femur (50%, n=8) and pelvis (25%, n=4). Fourteen (88%) cases underwent limb salvage/wide margin resection (n=13) or intralesional surgery (n=1). For all DCS, the median survival (IQR) was 46 months (12; 140), with both a five and ten-year probability of 32.1% (95% CI, 7.3% to 57.0%). At study conclusion, 81.3% (n=13) were deceased and 18.7% (n=3) were alive. Conclusions: Our findings confirm the poor prognosis of DCS patients, with a five-year estimate of 32%. Together with existing literature, our data might help enable future strategic recommendation of these patients.

What factors are important to new patients when selecting an orthopedic oncologist?

BACKGROUND: Understanding how patients choose a provider may improve the overall experience by identifying ways to tailor a clinical practice. AIM: To identify factors that patients consider important when choosing an orthopedic oncologist. METHODS: New patients presenting to an orthopedic oncology clinic within a tertiary academic medical center from January 2019 to August 2020 were invited to complete an anonymous survey. The questionnaire consisted of 27 items including a Likert-type assessment of the importance of selection factors. RESULTS: A total of 101 new patients with a median age of 66 years (range, 14 years to 91 years) responded. Most were referred by another doctor (n = 63, 62.4%), and of the referring providers, the most frequent specialty was orthopedic surgery (n = 32, 51%). Using a Likert-type scale with 1 representing 'least important' and 5 representing 'most important', the most important factor was the hospital reputation (mean, 4.65; SD, 0.85). Additional factors of importance were the number of years in practice (3.87 ± 1.3) and a primary care provider referral (3.71 ± 1.6). Patients younger than 40 years old found social media (P = 0.016) and internet presence (P = 0.035) of their surgeon to be more important than older patients. In contrast, older patients considered care within an academic center to be of greater importance than younger patients (P = 0.014). CONCLUSION: This investigation suggests a primary care referral, as well as hospital and physician reputation, are among the most important factors when selecting an orthopedic oncologist. Furthermore, social media utilization appears to be more important for younger patients.

High-Grade B-Cell Lymphoma of the Sciatic Nerve That Mimicked a Schwannoma: A Case Report.

CASE: A 41-year-old woman with a history of high-grade B-cell lymphoma status-post chemotherapy was evaluated for a painful left posterior thigh mass and significant weakness with sensory changes. Imaging revealed a benign-appearing mass consistent with schwannoma; however, intraoperative frozen section suggested high-grade B-cell lymphoma. Subsequent restaging studies revealed metastatic disease. CONCLUSION: A history of lymphoma warrants careful investigation of a posterior thigh mass, despite imaging findings consistent with nerve sheath tumor. Surgical removal of lymphomas of the nerve often proves futile, so these lesions are usually managed with medical treatment or radiation.

Does synovial sarcoma grade predict oncologic outcomes, and does a low-grade variant exist?

BACKGROUND AND OBJECTIVES: While historically aggressive, some synovial sarcomas (SS) are clinically indolent. This study sought to determine whether SS grade predicts oncologic outcomes and whether Grade 1 disease might exist. METHODS: Thirty-five cases from 2010 to 2019 were retrospectively reviewed. Clinicopathological data were analyzed and Kaplan-Meier assessed survival. RESULTS: The median patient age was 37 years (interquartile range: 28-51.5). The local control rate was 74.3%, and recurrence-free survival (RFS) was worse in positive versus negative margin resections (p = 0.023). The incidence of metastasis was 21.9% (n = 7) at a median 31 ± 31.7 months, and metastasis-free survival was 50.0% in Grade 3 SS versus 86.5% in Grade 2 (p = 0.026). Among a theoretical Grade 1 group, the overall survival (OS) and RFS profiles were improved compared to Grade 2 and 3 SS, respectively (p = 0.014 and p = 0.030). The Grade 1 group had a 15.8% (n = 3) metastatic rate and 80% 10-year survival. CONCLUSIONS: Tumor grade appears to predict outcomes in SS. A theoretical Grade 1 group showed improved OS and RFS versus Grades 2 and 3 SS, with metastatic rates and long-term survival resembling the historical literature for other low-grade soft tissue sarcomas. Our group continues to support the French Federation of Cancer Centers diagnostic strategy and NCCN treatment guidelines for SS.

Extraskeletal myxoid chondrosarcoma: A case series and review of the literature.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue sarcoma (STS) that accounts for less than 3% of all soft tissue tumors. The conventional treatment for primary EMC is wide local excision with or without radiation therapy. MATERIALS AND METHODS: This study was a retrospective review of all EMC cases treated within a single institution between 1992 and 2019. EMC was diagnosed using a combination of histologic morphology and immunostaining, with confirmatory fluorescent in situ hybridization. Overall survival (OS) and disease-specific survival (DSS) were defined using Kaplan-Meier analysis. RESULTS: Fifteen patients were evaluated, including 11 males and four females. The average age at presentation was 51.7 ± 20.4 years and the mean follow-up time was 61.5 months (range, 5-286 months). The average resected tumor size at largest dimension was 7.14 cm (range, 2.4-18.7). Twelve of fifteen (80%) patients underwent wide local excision, and nine of the twelve (75%) underwent local radiation therapy. The 1-, 5-, and 10-year OS was 80% (95% CI, 59.8-100), 72% (95% CI, 48.5-95.5), and 72% (95% CI, 48.5-95.5), respectively. The 1-, 5-, and 10-year DSS was 92.3% (95% CI, 77.8-100), 83.1% (95% CI, 61.5-100), and 83.1% (95% CI, 61.5-100), respectively. At last follow-up, 11 patients were alive and ten (90.9%) were disease free. CONCLUSIONS: Extraskeletal myxoid chondrosarcoma is a very rare STS most often seen in males and in the extremities. Our cohort was too small to provide meaningful statistical analysis; however, we observed lower rates of local recurrence in patients treated with radiation.

Tumors and Tumorlike Conditions of Which Every Orthopaedic Surgeon Should Be Aware.

All orthopaedic surgeons during the course of their career will likely encounter both benign and malignant musculoskeletal neoplasms. Given the rarity of these entities and the stress conferred by diagnosing a tumor or tumorlike condition, many orthopaedic surgeons may benefit from a review of the contemporary treatment of such patients. Whether in the outpatient clinic or following a high-energy trauma, special attention should be given to concerning signs and symptoms that will aid in the workup of children and adults with a possible tumor. A thorough and logical workup in this manner will often lead to a definitive diagnosis such as metastatic bone disease or perhaps a benign lesion. In these instances, the informed general orthopaedic surgeon or subspecialist may choose to treat the patient independently. However, if the workup is inconclusive or if the diagnosis is even questionably malignant, referral to an orthopaedic oncologist should be sought as to avoid pitfalls in diagnosis and treatment.

3D-Printed Cutting Guides for Resection of Long Bone Sarcoma and Intercalary Allograft Reconstruction.

The use of 3-dimensional (3D)-printed cutting guides for resection of long bone sarcoma is a novel technique. These 3D-printed guides provide a potential benefit over navigational or freehand osteotomy. We evaluated whether the use of 3D-printed cutting guides in the resection of long bone sarcoma affects margin status and rates of union compared with historical controls. In this study, we performed a retrospective review of a prospectively maintained surgical database and reviewed 6 patients who underwent limb salvage for long bone sarcoma. We collected and analyzed clinicopathologic and surgical data. Six (100%) cases recorded negative margins, with mean postoperative follow-up of 108 weeks (range, 8-211 weeks). Time (mean±SD) to bony union was 20.5±10.5 weeks. Nine of 12 (75%) cumulative (proximal and distal) osteotomy sites went on to achieve union, with a nonunion rate of 25% per osteotomy. One (33%) nonunion occurred after adjuvant radiation therapy. Long-term complications were limited to 2 (33.3%) patients overall who had implant failure according to the Henderson classification system, and there were zero local recurrences at the conclusion of the study. Our institution has successfully performed limb salvage surgery with patient-specific 3D-printed technology. We show high rates of negative margin resection and junctional union that align with and improve on earlier findings. [Orthopedics. 2022;45(1):e35-e41.].